Ocular Differential Diagnosis Compressed

Ocular Differential Diagnosis
Eighth Edition
Eighth Edition
Frederick Hampton Roy, M.D., F.A.C.S.

Little Rock, Arkansas
© 2007 by Frederick Hampton Roy
Published by F.E.P. International
All rights reserved. This book is protected by copyright. No part of this book may
be reproduced in any form or by any means, including photocopying, or utilized by any
information storage and retrieval system without written permission from the copyright
owner, except for brief quotations embodied in critical articles and reviews. Materials
appearing in this book prepared by individuals as part of their official duties as U.S.
government employees are not covered by the above-mentioned copyright.
Previous editions: 1972, 1977, 1982, 1987, 1992, 1997, 2002

Printed in the USA
ISBN X-XXXX-XXXX-X
Care has been taken to confirm the accuracy of the information presented and to
describe generally accepted practices. However, the author and publisher are not
responsible for errors or omissions or for any consequences from application of the
information in this book and make no warranty, expressed or implied, with respect to the
currency, completeness, or accuracy of the contents of the publication. Application of this
information in a particular situation remains the professional responsibility of the
practitioner.
The author and publisher have exerted every effort to ensure that drug selection
and dosage set forth in this text are in accordance with current recommendations and
practice at the time of publication. However, in view of ongoing research, changes in
government regulations, and the constant flow of information relating to drug therapy and
drug reactions, the reader is urged to check the package insert for each drug for any
change in indications and dosage and for added warnings and precautions. This is
particularly important when the recommended agent is a new or infrequently employed
drug.
Some drugs and medical devices presented in this publication have Food and
Drug Administration (FDA) clearance for limited use in restricted research settings. It is
the responsibility of the health care provider to ascertain the FDA status of each drug or
device planned for use in their clinical practice.
To Mary Michelle
To my children:
Nichols, Robert, Kimberly, Frederick, Jr., Charles, and Helena
To Dr. Arlington Krause,
molder and questioner in my early formative academic life
To Dr. Philip Lewis and Dr. Roger Hiatt
for guidance and direction
============= Contents =============
Preface ix
How to Use This Book xi
I. Regional Signs and Symptoms

1. Orbit 3
2. Lids 45
3. Lacrimal System 110
4. Extraocular Muscles 127
5. Conjunctiva 183
6. Globe 220
7. Sclera 231
8. Cornea 241
9. Intraocular Pressure 305
10. Anterior Chamber 329
11. Pupil 344
12. Iris 364
13. Lens 386
14. Vitreous 421
15. Retina 433
16. Choroid 526
17. Optic Nerve 559
18. Visual-Field Defects 604
II. General Signs and Symptoms

19. Visual Disturbance 619
20. Visual Complaint 644
21. Head Position 665
Subject Index 669
Preface
The first edition of Ocular Differential Diagnosis was published in 1972, and
various editions have been translated in Spanish, Turkish, Chinese, Portuguese, and
Italian. All previous editions have been my work. Dr. Gonzolo Murillo of La Paz,
Bolivia, helped in edition four and five with the diagnostic decision tables. In edition six,
section editors helped to standardize the language and made helpful suggestions. I feel
this is the best edition of Ocular Differential Diagnosis thus far. This text would not have
been possible without the superb efforts of Renee Tindall, Dr. Fernando Murillo, Angie
Brown, and Dr. Kae Chatman.
The Ocular Differential Diagnosis book provides comprehensive lists of causes
for symptoms or findings. Frequently more information is needed and hopefully Ocular
Syndromes and Systemic Diseases will furnish additional information to make a better
diagnosis. I hope the ophthalmologist and optometrist using this book will bring any
errors in this edition to my attention.
Frederick Hampton Roy, M.D.

Little Rock, Arkansas
How to Use This Book
This book can be used easily and quickly by following the directions presented
below.
1. If the sign or symptom relates to a particular region of the eye, turn to the table of
contents preceding this page to find the number of the page on which listings of the
signs and symptoms pertaining to the specific region begins. This latter page (or those
immediately following) will refer the user to that (or those) on which the various
causes of the condition are listed. For example, let us assume that the patient has
pigmentation of the cornea. The table of contents on page vii shows that the cornea
section begins on page 241. Turning to page 241 the user finds references to page 248
on which the causes of corneal pigmentation are listed according to type. In the subject
index, this topic is listed as Cornea, pigmentation of, 248.
2. If the symptom, such as binocular diplopia or night blindness, does not relate to a
particular region of the eye, look for it either in the subject index at the back of the
book or under General Signs and Symptoms beginning on page 619.
Various features of a disease may be crosschecked. For instance, a "pulsating
exophthalmos with orbital bruit and conjunctival edema" may be sought under orbit,
page 3, where the user of the book is referred to exophthalmos, page 4, and orbital
bruit, page 31, and under conjunctiva, page 183, where the user is referred to
conjunctival edema, page 202. The terms "exophthalmos," "orbital bruit" (under orbit,
bruit of) and "conjunctival edema" (under conjunctiva, edema of) may also be found in
the subject index. Terms such as "secondary glaucoma" are indexed under the noun,
e.g., glaucoma, secondary.
3. Following some of the differential diagnosis lists are diagnostic decision tables. These
tables list the history, physical signs, and laboratory tests that differentiate each
possible diagnosis. These can be identified in the subject index because they are
followed by a t.
Eighth Edition
PART I
Regional Signs and Symptoms
1
Orbit
CONTENTS
Pseudoproptosis 4
Exophthalmos 4
Syndromes and diseases associated with exophthalmos 8
Specific exophthalmos 11
Diagnostic tables 16
Up to one year 16
One to five years 17
Five to ten years 19
Ten to thirty years 21
Thirty to fifty years 22
Fifty to seventy years 24
More than seventy years 26
Pulsating exophthalmos 28
Recurrent exophthalmos 29
Enophthalmos 15
Intraorbital calcifications 31
Orbital bruit 31
Diagnostic table 32
Orbital emphysema 33
Orbital pain 33
Shallow orbits or diminished orbital volume 34
Pseudohypertelorism 35
Hypertelorism 35
Hypotelorism 38
Deep-set eyes 38
Prominent supraorbital ridges 38
Osteolysis of bony orbit 39
Fossa formation of orbit 39
Subperiosteal orbital hemorrhage 39
Orbital hemorrhage 40
Expansion of orbital margins 40
Hypertrophy of orbital bones 40
Expansion of optic canal 41
Small optic canals 41
Erosion of optic canal 42
Enlargement of superior orbital fissure 42
Narrowing of superior orbital fissure 43
Small orbit 43
Large orbit 43
Hematic orbital cysts 43
PSEUDOPROPTOSIS (APPEARANCE OF EXOPHTHALMOS)
*1. Asymmetry of bony orbits
2. Congenital cystic eyeball
*3. Contralateral enophthalmos (see p. 15)
4. Facial asymmetry as progressive facial hemiatrophy (Parry-Romberg syndrome)
5. Harlequin orbit (shallow orbit with arched superior and lateral wall) as with
hypophosphatasia
6. Hypoplastic supraorbital ridges as in trisomy (Edward syndrome)
7. Retraction of upper lid as with thyroid disease
8. Slight blepharoptosis as with Homer syndrome of contralateral eye
9. Shallow orbit as in Crouzon disease (craniofacial dysostosis)
10. Unilateral congenital glaucoma
11. Unilateral high-axial myopia
12. Unilateral secondary glaucoma resulting from ocular trauma during childhood
Newell FW. Ophthalmology: principles and concepts, 7th ed. St. Louis: CV Mosby,
1992.
Rootman J. Diseases of the orbit. Philadelphia: JB Lippincott, 1988.
EXOPHTHALMOS
1. Drugs, including the following:
adrenal cortex injection hydrocortisone methylthiouracil
aldosterone iodide and iodine oral contraceptives
beclomethasone solution and paramethasone
betamethasone compounds poliovirus vaccine
carbimazole levothyroxine prednisolone
cocaine liothyronine prednisone
cortisone liotrix propranolol
desoxycorticosterone lithium carbonate propylthiouracil
dexamethasone medrysone radioactive iodides
dextrothyroxine succinylcholine thyroglobulin
fludrocortisone meprednisone thyroid
fluorometholone methimazole triamcinolone
flu-prednisolone methylprednisolone vitamin A
2. Inflammation
A. Acute-orbital cellulitis
B. Acute suppurative-mucormycosis (diabetic or debility)
C. Allergic fungal sinusitis
D. Benign lymphoepithelial lesion (Mikulicz disease)
*E. Chronic (nongranulomatous-pseudotumor)
F. Chronic (granulomatous-tuberculosis, sarcoid (Schaumann syndrome), syphilis
(lues), parasites, aspergillosis)
G. Relapsing polychondritis
3. Injuries
A. Foreign body
B. Orbital hemorrhage
C. Orbital roof fracture
D. Secondary carotid cavernous sinus fistula
E. Thermal bums
4. Systemic disease
A. Acute intracranial hypertension
B. Amyloidosis (Lubarsch-Pick syndrome)
C. Chloroma
D. Cretinism (hypothyroidism)
E. Hydrocephalus and ventriculoperitoneal syndrome
F. Hypervitaminosis A
G. Hypophosphatasia (phosphoethanolaminuria)
*H. Thyroid disorder
I. Myasthenia gravis (Erb-Goldflam syndrome)
J. Obesity
5. Tumors
A. Cartilaginous tumors
(1) Cartilaginous hamartoma
(2) Chondroma
(3) Chondrosarcoma
(4) Mesenchymal chondrosarcoma
B. Cystic lesions
(1) Colobomatous cyst
(2) Dermoid cyst
(3) Hematocele
(4) Hydatid cyst
(5) Meningocele and meningoencephalocele
(6) Mucocele
(7) Optic nerve sheath cyst
(8) Simple epithelial cyst
(9) Teratoma
C. Fibrocytic tumors
(1) Fibroma
(2) Fibrosarcoma
(3) Fibrous histiocytoma
(4) Juvenile fibromatosis
(5) Nodular fasciitis
D. Histiocytic lesions
(1) Others
a. Juvenile xanthogranuloma (JXG, nevoxanthoendothelioma)
b. Sinus histiocytosis with massive lymphadenopathy
(2) Systemic histiocytoses (histiocytosis X) (Hand-Schüller-Christian
disease)
E. Inflammatory pseudotumor of orbit
(1) Ectopic cerebellar tissue in orbit
(2) Local, such as fungus or foreign body
(3) Systemic such as sarcoidosis syndrome (Schaumann syndrome) or
collagen disease
(4) Unknown cause
F. Lacrimal gland (fossa) lesions
(1) Epithelial tumors
a. Adenoid cystic carcinoma
b. Mucoepidermoid carcinoma
c. Pleomorphic adenocarcinoma (malignant mixed tumor)
d. Pleomorphic adenoma (benign mixed tumor)
(2) Nonepithelial lesions
a. Infectious
b. Inflammatory
c. Lymphoid and leukemia
d. Systemic (sarcoid)
G. Lipocytic and myxoid tumors
(1) Lipoma
(2) Liposarcoma
(3) Myxoid liposarcoma
(4) Myxoma
H. Lymphoid tumors and leukemias (excluding lacrimal gland lesions)
(1) Benign reactive lymphoid hyperplasia
(2) Burkitt lymphoma
(3) Lymphoblastic leukemia
(4) Myelogenous leukemia (granulocytic sarcoma)
(5) Non-Hodgkin lymphoma
I. Metastatic tumors of the orbit
(1) Malignant melanoma of skin
(2) Neuroblastoma (child)
(3) Other sites such as Ewing sarcoma
(4) Primary in breast (adult female)
(5) Primary in lung (adult male)
(6) Primary in prostate (adult male)
J. Nonepithelial lesions
(1) Benign reactive lymphoid hyperplasia
(2) Inflammatory pseudotumors (dacryoadenitis)
(3) Lymphoma
(4) Plasmacytoma
K. Optic nerve and meningeal tumors
(1) Juvenile pilocytic astrocytoma (optic nerve glioma)
(2) Meningioma
a. Primary optic nerve sheath
b. Secondary
(3) Malignant optic nerve glioma
L. Osseous and fibroosseous tumors
(1) Aneurysmal bone cyst
(2) Benign osteoblastoma
(3) Brown tumor of hyperparathyroidism
(4) Fibrous dysplasia (Albright syndrome)
(5) Giant cell granuloma
(6) Giant cell tumor (osteoclastoma)
(7) Infantile cortical hyperostosis
(8) Ossifying fibroma
(9) Osteoma
(10) Osteosarcoma
M. Peripheral nerve tumors
(1) Alveolar soft-part sarcoma
(2) Amputation neuroma
(3) Granular cell myoblastoma
(4) Neurilemoma
a. Benign
b. Malignant
(5) Neurofibroma
a. Plexiform
b. Solitary
(6) Paraganglioma (chemodectoma)
N. Primary melanocytic tumors
(1) Blue nevus
(2) Melanocytic hamartoma
(3) Melanotic progonoma (retinal tumor)
(4) Primary orbital melanoma
O. Rhabdomyoma and rhabdomyosarcoma
(1) Rhabdomyoma
(2) Rhabdomyosarcoma
P. Secondary orbital tumors from adjacent structures
(1) Conjunctival origin
a. Melanoma
Mucoepidermoid
c. Squamous cell carcinoma
(2) Eyelid origin
a. Basal cell carcinoma
b. Melanoma
c. Sebaceous carcinoma
d. Squamous cell carcinoma
(3) Intracranial origin
a. Astrocytoma
b. Meningioma
(4) Intraocular origin
a. Medulloepithelioma
b. Neurilemoma
c. Retinoblastoma
d. Uveal melanoma
(5) Nasopharyngeal origin
a. Angiofibroma
b. Carcinoma
c. Melanoma
(6) Paranasal sinus origin
a. Ethmoid sinus carcinoma
b. Inverting papilloma
c. Maxillary sinus carcinoma
d. Rhabdomyosarcoma
Q. Vasculogenic lesions
(1) Capillary hemangioma
(2) Cavernous hemangioma
(3) Hemangiopericytoma
(4) Hemangiosarcoma
(5) Kaposi sarcoma
(6) Lymphangioma
(7) Varices
(8) Vascular leiomyoma
(9) Vascular leiomyosarcoma
6. Vascular disorders
A. Allergic vasculitis
B. Angioedema (Quincke disease)
C. Arteriovenous aneurysm or varices
D. Arteriovenous fistula (varicose aneurysm)
E. Collagen disease-lupus erythematosus (Kaposi-Libman-Sacks syndrome),
periarteritis nodosa (Kussmaul disease), or dermatomucomyositis (Wagner-Unver-
richt syndrome)
F. Cranial arteritis
G. Thrombophlebitis
H. Scurvy causing bilateral orbital hemorrhage
Archer KF, et al. Orbital nonchromaffin paraganglioma. Ophthalmology 1989; 96:1659-

1666.
Carter K, et al. Ophthalmic manifestations of allergic fungal sinusitis. Am J OphthalmoI

1999; 127:189-195.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects. Woburn, MA:
Butterworth-Heinemann, 2001.
Luxenberg MN. Chloroma. Arch Ophthalmol 1991; 109:734-736.
Lyon DB, et al. Epithelioid hemangioendothelioma of the orbital bones. Ophthalmology

1993; 99:1773-1778.
Newman NJ, et al. Ectopic brain in the orbit. Ophthalmology 1986; 93:268-272.
Shields JA, et al. Classification and incidence of space-occupying lesions of the orbit.
Arch Ophthalmol 1984; 102:- 1606-1611.
Sloan B, et al. Scurvy causing bilateral orbital hemorrhage. Arch OphthalmoI 1999;
117:842.
SYNDROMES AND DISEASES ASSOCIATED WITH EXOPHTHALMOS
1. Actinomycosis
2. Albright syndrome (fibrous dysplasia)
3. Amyloidosis (Lubarsch-Pick syndrome)
4. Apert syndrome (sphenoacrocraniosyndactyly)
5. Arteriovenous fistula (varicose aneurysm)
6. Aspergillosis
7. Bacillus cereus
8. Bloch-Sulzberger disease (incontinentia pigment I)
9. Bonnet-Dechaume-Blanc syndrome (neuroretinoangiomatosis syndrome)
10. Bourneville syndrome (tuberous sclerosis)
11. Caffey syndrome (infantile cortical hyperostosis)
12. Carotid artery-cavernous sinus fistula
13. Clostridium perfringens
14. Coenurosis
15. Craniostenosis
16. Cretinism (hypothyroidism)
17. Crouzon disease (craniofacial dysostosis)
18. Cryptococcosis
19. Cushing syndrome (adrenocortical syndrome)
20. Dejean sign (orbital floor fracture)
21. de Lange syndrome (congenital muscular hypertrophy-cerebral syndrome)
22. Dermatomucomyositis (polymyositis dermatomyositis)
23. Dermoid
24. Diencephalic epilepsy syndrome (autonomic epilepsy syndrome)
25. Dirofilariasis
26. Dracontiasis (Guinea worm infection)
27. Engelmann syndrome (diaphyseal dysplasia)
28. Ewing sarcoma
29. Feer disease (infantile acrodynia)
30. Fibrosarcoma
31. Fibrous dysplasia (Albright syndrome)
32. Foix syndrome (cavernous sinus thrombosis)
33. Gardner syndrome
34. Grönblad-Strandberg syndrome (pseudoxanthoma elasticum)
35. Hallermann-Streiff-François syndrome (oculomandibulofacial dyscephaly)
36. Hand-Schüller-Christian disease (histiocytosis X)
37. Heerfordt syndrome (uveoparotid fever)
38. Hemangiomas
39. Herpes zoster
40. Hodgkin disease
41. Hollenhorst syndrome (chorioretinal infarction syndrome)
42. Homer syndrome (cervical sympathetic paralysis syndrome)
43. Hunter syndrome (MPS [mucopolysaccharidosis] II)
44. Hurler (MPS I-H) syndrome
45. Hutchinson disease (adrenal cortex neuroblastoma with orbital metastasis)
46. Hydatid cyst
47. Hydrocephalus chondrodystrophicus congenita (extreme hydrocephalus syndrome)
48. Hypertension
49. Hyperthyroidism (Basedow syndrome)
50. Hypervitaminosis A
51. Hypophosphatasia (phosphoethanolaminuria)
52. Jansen disease (metaphyseal dysostosis)
53. JXG (nevoxanthoendothelioma)
54. Kleeblattschädel syndrome (cloverleaf skull)
55. Leiomyoma
56. Leopard syndrome (multiple lentigines syndrome)
57. Leprechaunism
58. Leukemia
59. Linear nevus sebaceous of Jadassohn
60. Lupus erythematosus (Kaposi-Libman-Sacks syndrome)
61. Lymphoid hyperplasia
62. Lymphangioma
63. Lymphosarcoma
64. Melnick-Needles syndrome (osteodysplasty)
65. Meningioma
66. Mikulicz syndrome (dacryosialoadenopathy)
67. Möbius disease (congenital paralysis of sixth and seventh nerves)
68. Mucocele
69. Mucormycosis
70. Multiple myeloma
71. Mumps
72. Myasthenia gravis (Erb-Goldflam syndrome)
73. Neurilemoma
74. Noonan syndrome (male Turner syndrome)
75. Osteopetrosis (Albers-Schönberg syndrome)
76. Paget syndrome (osteitis deformans)
77. Periarteritis nodosa (Kussmaul syndrome)
78. Periocular and ocular metastatic tumors
79. Pfeiffer syndrome
80. Pierre-Robin syndrome (micrognathia-glossoptosis syndrome)
81. Progeria (Hutchinson-Gilford syndrome)
82. Pyknodysostosis
83. Quincke disease (angioedema)
84. Relapsing polychondritis
85. Retinoblastoma
86. Rhabdomyosarcoma
87. Rochon-Duvigneaud syndrome (superior orbital fissure syndrome)
88. Rollet syndrome (orbital apex-sphenoidal syndrome)
89. Sarcoidosis syndrome (Schaumann syndrome)
90. Scaphocephaly syndrome
91. Scheie syndrome (MPS I-S)
92. Scurvy (avitaminosis C)
93. Sebaceous gland carcinoma
94. Seckel syndrome (bird-headed dwarf syndrome)
95. Sézary syndrome (mycosis fungoides syndrome)
96. Shy-Gonatas syndrome (orthostatic hypotension syndrome)
97. Siegrist sign (pigmented choroidal vessels)
98. Silverman syndrome (battered baby syndrome)
99. Sphenocavernous syndrome
100. Streptococcus
101. Sturge-Weber syndrome (encephalofacial angiomatosis)
102. Syphilis (lues)
103. Thermal bums
104. Trichinellosis
105. Trisomy syndrome (Edward syndrome)
106. Tuberculosis
107. Turner syndrome (gonadal dysgenesis)
108. von Hippel-Lindau syndrome (retinocerebral angiomatosis)
109. von Recklinghausen disease (neurofibromatosis)
110. Wegener syndrome (Wegener granulomatosis)
Goodman RM, Gorlin RJ. The face in genetic disorders. 51. Louis: CV Mosby, 1970.
Miller NR, et al. Lytic Paget disease as a cause of orbital cholesterol granuloma. Arch
Ophthalmol I999; 117: 1084-1085.
Roy PH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott
Williams & Wilkins, 2002.
SPECIFIC EXOPHTHALMOS
1. Age
A. Newborn-most common
*(1) Orbital sepsis
(2) Orbital neoplasm including congenital malignant teratoid neoplasm
B. Neonatal-osteomyelitis of the maxilla
C. Early childhood (up to 1 year of age-most common)
*(1) Dermoid
*(2) Hemangioma
(3) Dermolipoma
(4) Histiocytosis X including Hand-Schüller-Christian disease
*(5) Orbital extension of retinoblastoma
D. One to five years-most common
*(1) Dermoid
(2) Metastatic neuroblastoma
(4) Epithelial cyst, such as sebaceous cyst and epithelial inclusion cyst
(5) Glioma of optic nerve
(6) Sphenoid wing meningioma
*(7) Orbital extension of retinoblastoma
(9) Metastatic embryonal sarcoma
*(10) Hemangioma
E. Five to ten years-most common
(1) Pseudotumor
(2) Orbital extension of retinoblastoma
(3) Malignant lymphomas and leukemias
*(4) Dermoid
*(5) Hemangioma
(6) Meningioma
(9) Orbital hematoma
F. Ten to thirty years-most common
*(1) Pseudotumor
(2) Mucocele
(3) Meningioma
*(4) Endocrine ophthalmopathy (thyroid-related ophthalmopathy)
(5) Lacrimal gland tumor
(7) Dermoid
(8) Hemangioma
(9) Peripheral nerve tumors
(10) Undifferentiated sarcomas
(11) Osteoma
G. Thirty to fifty years-most common
*(1) Pseudotumor -
(2) Mucocele
*(4) Hemangioma
(6) Lacrimal gland tumors
(7) Rhinogenic carcinoma
(8) Malignant melanoma
(9) Osteosarcoma
(10) Fibrosarcoma
(11) Metastatic carcinoma
(12) Meningioma
(13) Dermoid
H. Fifty to seventy years-most common
*(1) Pseudotumor
*(2) Mucocele
*(3) Malignant lymphomas and leukemias
(4) Dermoid
(5) Carcinoma of palpebral or epibulbar origin
*(6) Meningioma
(8) Lacrimal gland tumor
(9) Osteosarcoma
(10) Fibrosarcoma
(11) Undifferentiated sarcoma
(13) Osteoma
(15) Neurofibroma
(16) Hemangioma
I. More than seventy years-most common
(1) Melanoma
(2) Pseudotumor
*(3) Lymphoma
*(4) Metastatic tumor
(5) Basal cell carcinoma
(6) Mucocele
2. Unilateral exophthalmos-most common
A. Anatomical conditions
(1) Unilateral myopia of high degree
(2) Defects in the vault of the orbit: meningocele, encephalocele,
hydroencephalocele
(3) Exophthalmos associated with arterial hypertension
(4) Recurrent exophthalmos from retrobulbar hemorrhage, lymphangioma
(5) Intermittent exophthalmos associated with venous anomalies within
the cranium
(6) Disease of the pituitary gland; meningiomas involving sphenoid ridge
*(7) Unilateral exophthalmos associated with endocrine or thyroid-related
ophthalmopathy
B. Traumatic conditions
(1) Contralateral floor fracture with enophthalmos
(2) Fracture of the orbit with retrobulbar hemorrhage
(3) Laceration and rupture of the tissues of the orbit and the extraocular
muscles
(4) Intracranial trauma sustained at birth; aneurysm in orbit
(5) Pulsating exophthalmos from carotid-cavernous aneurysm
(6) Spontaneous retrobulbar hemorrhage as seen in whooping cough
(7) Chronic subdural hematoma bulging into orbit
(8) Posterior exophthalmos (orbital apex lesion)
a. Pseudotumor
b. Malignant tumor
c. Benign tumor
d. Vascular disease
e. Infection
C. Inflammatory conditions
(1) Retrobulbar abscess and cellulitis
(2) Thrombophlebitis of the orbital veins
(3) Cavernous sinus thrombosis
(4) Erysipelas (St. Anthony fire)
(5) Tenonitis
(6) Periostitis (syphilitic or tuberculous)
(7) Orbital mucocele, pyocele; cholesteatoma
(8) Orbital exostosis
(9) Paget disease with hyperostosis
(10) Actinomycosis, trichinosis, mycotic pseudotumor
(11) Herpes (HSV-l) with acute retinal necrosis
D. Disease of blood, lymph, and hematopoietic system
(1) Rickets (avitaminosis D)
(2) Scurvy (avitaminosis C)
(3) Hemophilia (factor VIII deficiency)
(4) Lymphosarcoma
(5) Chloroma
(6) Hodgkin disease
E. Space-taking lesions
(1) Vascular anomalies
a. Congenital orbital varix (young patient with systemic
abnormalities)
b. Cavernous hemangioma (middle age)
c. Capillary hemangioma (young children) Kasabach-Merrit
syndrome
d. Lymphangiomas
(2) Orbital tumors: pseudotumors, orbital cysts, meningocele,
lymphangioma, orbital meningioma, lacrimal gland tumor, sarcoma,
metastatic carcinoma, metastatic adrenal tumors, osteomas arising in the
accessory nasal sinuses, tumors of the nasopharynx (benign and
malignant)
(3) Intracranial tumor with orbital extension including chordoma and
meningioma
F. Unilateral exophthalmos in children
(1) Inflammation
(2) Vascular disorders
(3) Neoplasms
(4) Metabolic diseases
(5) Developmental anomalies
(6) Others
*(7) Orbital cellulitis
3. Bilateral exophthalmos-most common
*A. Thyroid or endocrine ophthalmopathy
B. Orbital myositis (owing to causes other than thyroid dysfunction)
C. Cavernous sinus thrombosis (Foix syndrome)
D. Metastatic neuroblastoma
E. Hand-Schüller-Christian disease (histiocytosis X)
F. Crouzon disease (craniofacial dysostosis)
G. Paget disease (osteitis deformans)
4. Type proptosis-most common
A. Straightforward-glioma of optic nerve, intraconal cavernous hemangioma
B. Down and temporal-mucocele of frontal sinus
C. Down and nasal-lacrimal gland lesion
D. Downward-tumor of roof of orbit
E. Upward-tumor of floor of orbit
5. Transient exophthalmos
*A. Orbital varices
B. Orbital varices with intracranial extension
C. Arteriovenous malformations
D. Cavernous hemangioma
E. Intraorbital arteriovenous malformation
6. Pulsating exophthalmos-most common
*A. Carotid-cavernous fistula
B. von Recklinghausen disease associated with bony defect of skull
C. Large frontal mucocele
D. Meningoencephalocele
E. Blow-in fracture of roof of orbit
F. Neurofibromatosis
G. Fistula
H. Malignancies
I. Mucoceles
J. Orbital varix
K. Dermoid cysts
L. Aneurysm
7. Recurrent exophthalmos
A. Recurrent orbital inflammation (pseudotumor) or hemorrhage
B. Orbital cysts that rupture
C. Lymphangioma (children)
D. Syndrome of intermittent exophthalmos-congenital venous malformations of
the orbit: venous angioma and orbital varix
E. Temporal lobe tumor with orbital extension
F. Neurofibromatosis
G. Vascular neoplasm
8. Intermittent exophthalmos
A. Orbital varices
B. Recurrent hemorrhage
C. Vascular neoplasm
D. Lymphangioma
9. Exophthalmos associated with conjunctival chemosis, restricted movement of eyes
because of pain-pseudotumor
10. Exophthalmos in an acutely ill patient-cavernous sinus thrombosis
11. Exophthalmos associated with engorged conjunctival episcleral vessels
A. Nonpulsating-cerebral arteriovenous angioma, ophthalmic vein thrombosis, or
cavernous sinus thrombosis
B. Pulsating exophthalmos-carotid-cavernous sinus fistula
12. Exophthalmos associated with a palpable mass in region of the lacrimal gland
A. Primary inflammatory exophthalmos
B. Neoplasm
C. Sarcoidosis syndrome (Schaumann syndrome)
D. Hodgkin disease
13. Exophthalmos in patient with uncontrolled diabetes, usually with acidosis, who
develops unilateral lid edema, ptosis, internal and external ophthalmoplegia, proptosis,
and severe vision loss-orbital mucormycosis
14. Exophthalmos in an infant with ecchymosis of the eyelids
A. Metastatic neuroblastoma
B. Orbital leukemia infiltration
15. Bilateral exophthalmos from bilateral orbital pseudotumor
A. Eosinophilic granuloma
B. Retroperitoneal fibrosis
C. Myasthenia gravis (Erb-Goldflam syndrome)
Henderson JW. Orbital tumors. New York: Thieme-Stratton. 1980.
Jones IS, Jakobiec FA. Diseases of the orbit. Philadelphia: Harper & Row, 1979.
Katz SE, et al. Combined venous lymphatic malformations of the orbit (so-called
lymphangiomas). Ophthalmology 1998; 105: 176-184.
Klapper SR, et al. Orbital involvement in allergic fungal sinusitis. Ophthalmology 1997;
104:2094-2100.
Krohel GB, et al. Orbital disease: a practical approach. Stewart WB, Chavis RM, eds.
New York: Grune & Stratton, 1981.
Moin M, et al. Spontaneous hemorrhage in an intraorbital arteriovenous malformation.
Ophthalmology 2000; 107: 2215-2219.
Nicholson DH, Green WR, eds. Pediatric ocular tumors. New York: Masson, 1981.
Steinkuller PG, Font RL. Congenital malignant teratoid neoplasm of the eye and orbit.
Ophthalmology 1997; 104: 38-42.
Tornerup NR, et al. HV-1 induced acute retinal necrosis syndrome presenting with severe
inflammatory orbitopathy proptosis and optic nerve involvement. Ophthalmology 2000;
107:397-401.
Wright JE, et al. Orbital venous anomalies. Ophthalmology 1 997; 104:905-913.
ENOPHTHALMOS
1. Senility (common)
2. Wasting diseases-loss of orbital fat
*3. Injury-blowout fracture of floor of orbit (most common)
4. Orbital varices-transient exophthalmos with fat atrophy
Extracted Tables:
Table Exophthalmos (Up to 1 year)

Table Exophthalmos (1-5 years)
Table Exophthalmos (more than 70 years)
Table Pulsating exophthalmos (most common)
Table Recurrent exophthalmos
5. Chronic or severe liver or gallbladder disease (usually in right eye owing to increased
tone of orbicularis muscle and extraocular muscles)
6. Iatrogenic
A. Orbital decompression
B. Sinus surgery
7. Superior sulcus deformity
A. Traumatic bony loss
B. Atrophy of the orbital tissues
C. Levator detachment with ptosis
D. Migration of muscle cone implant
E. Herniated orbital fat secondary to an orbital fracture
8. Associated syndromes
A. Arthrogryposis (amyoplasia congenital)
B. Babinski-Nageotte syndrome (medullary tegmental paralysis)
C. Cestan-Chenais syndrome (lesion in the lateral portion of medulla oblongata)
D. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
E. Craniocervical syndrome (whiplash injury
F. Cretinism (hypothyroidism)
G. Cryptophthalmia syndrome
H. Dejean syndrome (orbital floor syndrome)
I. Dejerine-Klumpke syndrome (thalamic hyperesthetic anesthesia)
J. Duane retraction syndrome
K. Freeman-Sheldon syndrome (craniocarpotarsal dysplasia)
L. General fibrosis syndrome
M. Greig syndrome (ocular hypertelorism syndrome)
N. Hemifacial microsomia syndrome (François-Haustrate syndrome)
O. Horner syndrome (cervical sympathetic paralysis syndrome)
P. Klippel-Trenaunay-Weber syndrome (angioosteohypertrophy syndrome)
Q. Krause syndrome (encephaloophthalmic syndrome)
R. Maple syrup urine disease (branched chain ketoaciduria)
S. Morquio syndrome (MPS IV)
T. Naffziger syndrome (scalenus anticus syndrome)
U. Pancoast syndrome (superior pulmonary sulcus syndrome)
V. Parry-Romberg syndrome (progressive facial hemiatrophy)
W. Passow syndrome (Bremer status dysraphicus)
X. Raeder syndrome (paratrigeminal paralysis)
Y. Retroparotid space syndrome
Z. Silent sinus syndrome
AA. Vernet syndrome (jugular foramen syndrome)
BB. von Herrenschwand syndrome (sympathetic heterochromia)
CC. Wallenberg syndrome (dorsolateral medullary syndrome)
9. Apparent enophthalmos with horizontal conjugate gaze
10. Metastatic adenocarcinoma of orbit (cicatricial)
11. Neurofibromatosis: pulsating enophthalmos
12. Typhoid fever (abdominal typhus)
Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Saunders, 1994:1881-2095.
Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology 1984; 91:229-

237.
Roy FH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott
Stasior OG, Roen JL. Traumatic enophthalmos. Ophthalmology 1982; 89:1267.
INTRAORBITAL CALCIFICATIONS
1. Calcification of more irregular configuration and texture

A. Cysticercosis
B. Orbital hematoma
C. Plexiform neurofibroma
D. Toxoplasmosis
E. Tuberculosis
2. Calcification of orbital vessels
A. Atheromatous plaque
B. Monkeberg sclerosis
C. Secondary to metabolic-endocrine disturbances such as hyperparathyroidism or
hypervitaminosis
D. Band-shaped keratopathy
3. Chronic inflammatory and parasitic disease of the orbit
4. Hemangiopericytoma
5. Intraocular calcifications following
A. Congenital deformity
B. Malignant lacrimal gland tumor
C. Recurrent iritis and keratitis
D. Retinal detachment
E. Trauma (perforating, nonperforating, or surgical)
6. Intraocular sarcoma
7. Mucocele
8. Myositis ossificans
9. Orbital phleboliths: helical form in veins-smooth, round, or oval
10. Organized hematomas of the orbit
* 11. Retinoblastoma
12. Retrolental fibroplasia
13. Sites of intraocular calcification
A. Cyclitic membrane
B. Lens
C. Peripapillary choroid
D. Posterior pole to ora serrata in region of choroid and pigment epithelium
E. Retina
F. Vitreous

Saunders, 1994: 1881-2095.
Basta LL, et al. Focal choroidal calcification. Ann Ophthalmol 1981; 13:447-450.
Garrity JA, Kennerdell JS. Orbital calcification associated with hemangiopericytoma. Am

J Ophthalmol 1986; 102: 126.
Zizmor J, Lombardi G. Atlas of orbital radiology. Birmingham: Aesculapius, 1973.
ORBITAL BRUIT (NOISE HEARD OVER ORBIT WITH STETHOSCOPE)

Extracted Table Orbital bruit (noise heard over orbit with stethoscope)
1. Bilateral
A. Hyperthyroidism
B. Severe anemias
2. Unilateral
*A. Abnormal communication in the cavernous sinus (i.e., bilateral carotid-
cavernous sinus)
B. Aneurysmal angioma of orbit or fundus such as in Wyburn-Mason syndrome
(Bonnet- Dechaume-Blanc syndrome)
C. Arteriovenous aneurysm (arteriovenous fistula)
D. Intermittent or pulsating exophthalmos
E. Stenosis of carotid artery including thrombosis, sclerosis, or external pressure
such as that due to an outer-ridge sphenoidmeningioma

Saunders, 1994: 1881-2095.
Grobb WE, et al. Facial hamartomas in chi1dten: neurofibroma, lymphangioma, and

hemangiomia. Plast Reconstr Surg 1980; 66:509.
Kushner FH. Carotid-cavernous fistula as a complication of carotid endarterectomy. Ann

Ophthalmol 1981; 13:979.
Lloyd GA. Vascular anomalies in the orbit: C T and angiographic diagnosis. Orbit 1982;
1:45.
ORBITAL EMPHYSEMA (AIR FOUND IN ORBITAL TISSUES AND ADNEXA

USUALLY DEMONSTRABLE BY PALPATION)
*1. Due to fracture of ethmoid sinuses or orbital floor

2. Following forceful blowing of nose
3. Injury from compressed air
4. Orbital cellulitis and abscess with gas formation by infecting organism
5. Osteomyelitis and infected sinus with fistulous communication with gas formation by
infecting organism
6. Resulting from use of high-speed dental drill and air-water spray during oral operation
7. Subconjunctival emphysema seen with mechanical ventilation
Buckley MJ, et al. Orbital emphysema causing vision loss after a dental extraction. J Am
Dent Assoc 1990; 120: 421-422.
Hunts JH, et al Orbital emphysema: staging and acute management. Ophthalmology

1994; 101:960-966.
Zimmer-Galler IE, Bartley GB. Orbital emphysema: case reports and review of the
literature. Mayo Clin Proc 1994; 69:115-121.
ORBITAL PAIN
1. Acute dacryoadenitis
2. Amputation neuroma of the orbit
3. Associated syndromes
A. Cavernous sinus thrombosis syndrome
B. Charlin syndrome (nasal nerves syndrome)
C. Erysipelas
D. Ophthalmoplegic migraine syndrome
E. Raeder syndrome (paratrigeminal paralysis)
F. Tolosa-Hunt syndrome (painful ophthalmoplegia).
4. Break-bone fever (dengue fever)
6. Eye strain from uncorrected errors of refraction
7. Myositis
A. Collagen diseases
B. Infectious myositis
C. Trichinosis
8. Orbital cellulitis or abscess
9. Orbital periostitis because of injury, tuberculosis, syphilis, extension of sinus disease,
or other conditions
*10. Pseudotumor or tumor of the orbit-pain infrequently present
11. Retrobulbar neuritis
12. Trauma
13. Tumors of cerebellopontine angle, frequent lesion of seventh nerve

Saunders, 1994: 1881-2095.
Bullen CL, Younge BR. Chronic orbital myositis. Ophthalmology 1982; 89:1749.
Lanzino G, et al. Orbital pain and unruptured carotid-posterior communicating artery

aneurysms: the role of sensory fibers of the third cranial nerve. Acta Neurochir (Wien)
1993; 120:7-11.
Roy FR. Ocular syndromes and systemic diseases. 3rd ed. Philadelphia: Lippincott
SHALLOW ORBITS OR DIMINISHED ORBITAL VOLUME (ILLUSION OF

PROPTOSIS OR GLAUCOMA)
1. Aminopterin-induced syndrome
2. Apert syndrome (acrocephalosyndactyly)
3. Carpenter syndrome
4. Cerebrohepatorenal syndrome (Smith-Lemli-Opitz syndrome)
5. Craniostenosis
7. Diseases of nasal passages and sinuses
A. Dentigerous cysts
B. Fibrous dysplasia (Albright syndrome)
C. Hypoplasia of maxilla associated with chronic maxillary sinusitis
D. Rhinoscleroma
8. Dubowitz syndrome
9. Early enucleation of eye
*10. Familial hypoplasia of orbital margin
11. Frontometaphyseal dysplasia (FMD)
12. Hyperostosis (hypertrophy of orbital bones)
13. Hypophosphatasia-harlequin orbit (shallow orbit with arched superior and lateral
wall)
14. Kleeblattschädel syndrome
15. Lateral displacement of medial orbital wall by hypertrophic polypoid nasal sinus
disease
16. Marshall-Smith syndrome
17. Oculoauriculovertebral dysplasia (Goldenhar syndrome)
18. Osteogenesis imperfecta (van der Hoeve syndrome)
19. Radiation injury of bone
20. Robert syndrome (pseudothalidomide syndrome)
21. Saethre-Chotzen syndrome
22. Secondary to fracture
23. Stanesco dysostosis syndrome
24. Trisomy 13-(trisomy D) (Patau syndrome)
25. Trisomy (Edward syndrome)
26. Zellweger syndrome
27. 6q- D syndrome
28. 9p- syndrome
Cursiefen C, et al. Adenoma of the nonpigmented ciliary epithelium mimicking a

malignant melanoma of the iris. Arch Ophthalmol 1999; 1l7:113-118.
Roy FR. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott
Smith DW. Recognizable patterns of human malformation, 4th ed. Philadelphia: WB

Saunders, 1988.
PSEUDOHYPERTELORISM (ILLUSION OF INCREASED DISTANCE

BETWEEN BONY ORBITS AND INCREASED INTERPUPILLARY DISTANCE)
1. Blepharophimosis
*2. Epicanthal skin folds
3. Exotropia
*4. Flat nasal bridge of nose
5. Increased distance between the inner canthi (te1ecanthus)
6. Widely spaced eyebrows
DeMyer W. The median cleft face syndrome. Neurology 1967; 17:961.
HYPERTELORISM (INCREASED DISTANCE BETWEEN BONY ORBITS AND

INCREASED INTERPUPILLARY DISTANCE)
1. Aarskog syndrome (faciodigitogenital syndrome)

2. Acrocollosal syndrome
3. Acrodysostosis syndrome
4. Albers-Schönberg disease (osteopetrosis)
5. Aminopterin-induced syndrome
*6. Apert syndrome (acrodysplasia)
*7. Association of hyperte1orism, microtia, and facial clefting
*8. Baraitser- Winter syndrome
9. BBB syndrome (hypertelorism-hypospadias syndrome)
10. Blatt syndrome (cranioorbitoocular dysraphia)
11. Blepharonosofacial syndrome
12. Camptomelic dysplasia syndrome
13. Carpenter syndrome (acrocephalopolysyndactyly II)
14. Cat's-eye syndrome (Schachenmann syndrome)
15. Cerebral gigantism (Sotos syndrome)
16. Cerebrohepatorenal syndrome (Zellweger)
17. Cherubism
18. Chromosome partial long-arm deletion syndrome (de Grouchy syndrome)
19. Chromosome partial short-arm deletion syndrome [monosomy partial (short-arm)
syndrome]
20. Chromosome short-arm deletion
21. Chondrodystrophia calcificans congenita (Conradi syndrome)
22. Cleft lip and palate sequence
23. Clefting, ectropion, and conical teeth syndrome
24. Cleidocranial dysostosis syndrome
25. Coffin-Lowry syndrome
26. Congenital hemihypertrophy
27. Craniocarpotarsal syndrome (Freeman-Sheldon syndrome)
28. Craniocleidodysostosis syndrome (Marie-Sainton syndrome)
29. Craniosynostosis-radial aplasia (Baller-Gerold syndrome)
31. Cri-du-chat syndrome (Cry of the cat syndrome)
33. Cryptophtha1mos syndrome
34. Curtius syndrome (ectodermal dysplasia with ocular malformations)
35. Diamond-Blackfan syndrome
36. DiGeorge sequence
37. Down syndrome (mongolism)
38. Dubowitz syndrome (dwarfism-eczema-peculiar facies)
39. Duplication 14Q syndrome
40. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
42. 18P syndrome
43. Faciooculoacousticorenal syndrome
44. Familial characteristic
45. Familial metaphyseal dysplasia (Pyle disease)
46. Fetal alcohol syndrome
47. Fetal aminopterin effects
48. Fetal hydantoin effects
49. Fish odor syndrome
50. 4Q syndrome
51. Frontonasal dysplasia syndrome (median cleft face syndrome)
52. Frontal encephaloceles
53. Gorlin syndrome (orodigitofacial dysostosis)
54. Greig syndrome (hypertelorism)
55. Haney-Falls syndrome (congenital keratoconus posticus circumscriptus)
56. Holt-Dram syndrome
57. Hurler syndrome (MPS I-H)
58. Hydrocephalus
59. Hypomelanosis of Ito syndrome (systematized achromic nevus)
60. Ichthyosis (collodion baby)
61. Infantile gigantism
62. Infantile hypercalcemia with supravalvular aortic stenosis (Williams-Beuren
syndrome)
63. Iris dysplasia-hypertelorism-psychomotor retardation syndrome
64. Jacobs syndrome (triple X syndrome)
65. KBG syndrome (initials of family studied)
66. Kleeblattschädel syndrome (extreme hydrocephalus syndrome)
67. Klein syndrome
68. Klinefelter XXY syndrome (gynecomastia-aspermatogenesis syndrome)
69. Klippel-Feil syndrome (synostosis cervical vertebrae)
70. Larsen syndrome
71. Leprechaunism
72. Lissencephalia (Miller-Dieker syndrome)
73. Little syndrome (nail-patella syndrome)
74. Mandibulofacial dysostosis (Franceschetti syndrome)
75. Maple syrup urine disease (branched-chain ketoaciduria)
76. Marfan syndrome (arachnodactyly-dystrophia-mesodermalis congenita)
77. Marshall-Smith syndrome
78. Meckel-Gruber syndrome
79. Melnick -Needles syndrome (osteodysplasty)
80. Metaphyseal dysostosis (Jansen disease)
81. McFarland syndrome
82. Morquio-Ullrich syndrome (MPS IV)
83. Multiple basal cell nevi (Godin-Goltz syndrome)
84. Multiple lentigines syndrome (Leopard syndrome)
85. Myelomeningocele-Chiari malformations
87. Oculodentodigital syndrome
88. Oculomandibulofacial dyscephaly (Hallermann-Streiff syndrome)
89. Optic nerve hypoplasia
90. Orofaciodigital (OFD) type I and type II (Mohr syndrome)
91. Osteogenesis imperfecta (van der Hoeves syndrome)
92. Otopalatodigital syndrome (OPD syndrome)
93. Pallister-Killian syndrome
94. Pena-shokeir type I syndrome
96. Potter syndrome (renofacial syndrome)
97. Ring B chromosome
98. Ring chromosome
99. Rieger syndrome (dysgenesis mesostromalis)
100. Robert syndrome (pseudothalidomide syndrome)
101. Robinow syndrome (fetal face syndrome)
102. Saethre-Chotzen syndrome (acrocephalosyndactyly type III)
103. Sjögren-Larson syndrome (oligophrenia-ichthyosis-spastic diplegia syndrome)
104. Sprengel syndrome
105. Traumatic nasoorbital fracture
106. Triploidy syndrome
107. Trisomy syndrome
108. Trisomy 6 q syndrome
109. Trisomy 9q syndrome
1l0. Trisomy 13- (Patau syndrome)
111. Trisomy 17p syndrome
113. Turner-Bonnevie-Ullrich-Nielsen syndrome
115. Waardenburg syndrome (embryonic fixation syndrome)
116. Weaver syndrome
117. Williams syndrome
118. XXXX syndrome
119. XXXXX syndrome
120. XXXXY syndrome
121. 4p- syndrome (Wolf syndrome)
122. 4p- D syndrome
123. 6p- D syndrome
124. 9p- syndrome
125. 10q- syndrome
126. 13q- syndrome
127. 18q- syndrome
Pallotta R. Iris coloboma ptosis, hypertelorism, and mental retardation: a new syndrome
possibly localized on chromosome 2. J Med Genet 1991; 28:342-344.
Seaver LH, Cassidy SB. New syndrome: mother and son with hypertelorism,
downslanting palpebral fissures, malar hypoplasia, and apparently low-set ears associated
with joint and scrotal anomalies. Am J Med Genet 1991; 41: 405-409.
HYPOTELORISM (DECREASED DISTANCE BETWEEN BONY ORBITS AND

DECREASED INTERPUPILLARY DISTANCE)
1. Arrhinencephaly (holoprosencephaly)
2. Cebocephalia
3. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
4. Coffin-Siris syndrome
5. Ethmocephalus
*6. Familial
7. François diencephalic syndrome (Hallerman-Streiff syndrome)
8. Goldenhar syndrome (oculoauriculovertebral dysplasia)
9. Maternal phenylketonuria fetal effects
10. Meckel-Gruber syndrome
11. Median cleft lip (frontonasal dysplasia syndrome)
12. Median philtrum-premaxilla anlage
13. Ocular-dental-digital dysplasia (Meyer-Schivickerath and Weyers syndrome)
14. Ring syndrome
15. Trigonocephaly (C syndrome, Opitz trigonocephaly syndrome)
16. Trisomy 13- (Patau syndrome, trisomy D syndrome)
18. Trisomy (Down syndrome, mongolism)
21. Wolf syndrome (monosomy partial syndrome)
22. 5p- D syndrome
23. 18p- syndrome
Evans DG. Dominantly inherited microcephaly, hypotelorism and normal intelligence.

Clin Genet 1991; 39: 178-180.
Judisch GF, et al. Orbital hypotelorism. Arch Ophthalmol 1984;102:995-997.

Richieri-Costa A, et al. Mental retardation, microbrachycephaly, hypotelorism, palpebral
ptosis, thin/long face, cleft lip, and lumbosacral/pelvic anomalies. Am J Med Genet 1992;
43:565-568.
DEEP-SET EYES

2. Craniocarpotarsal syndrome (Freeman-Sheldon syndrome)
3. Familial
4. Marfan syndrome (dolichostenomelia-arachnodactylyhyperchon-droplasia-dystrophia
mesodermalis congenita)
5. Mesodermal dysmorphodystrophy (Weill- Marchesani syndrome)
6. Oculocerebrorenal syndrome (Lowe syndrome)
7. Pyknodysostosis
8. Syndrome of blepharophimosis with myopathy
Aita JA. Congenital facial anomalies with neurologic defects. Springfield, IL: Charles C
Thomas, 1969.
PROMINENT SUPRAORBITAL RIDGES
1. Apert syndrome (acrocephalosyndactylism syndrome)

2. Basal cell nevus syndrome (Godin-Goltz syndrome)
3. Cleidocranial dysostosis (Marie-Sainton syndrome)
4. Congenital lipodystrophy
5. Congenital syphilis (congenital lues)
6. Ectodermal dysplasia (Curtius syndrome)
*7. Frontometaphyseal dysplasia
9. Marfan syndrome (arachnodactyly-dystrophia mesodermalis congenita)
10. Otopalatodigital syndrome (Taybi syndrome)
11. Pyle metaphyseal dysplasia syndrome
Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WE

Saunders, 1994: 1881-2095.
OSTEOLYSIS OF BONY ORBIT

1. Autoimmune diseases, such as Wegener granulomatosis
2. Congenital
3. Hyperparathyroidism
4. Injury, such as blowout fracture of orbital floor
5. Meningocele and encephalocele of orbit
6. Metastasis from remote primary neoplasms
7. Primary orbital disease
A. Infectious, including tuberculosis and syphilis
B. Neoplastic, including neurofibroma and lacrimal gland tumor
C. Cystic, including dermoid and epidermoid cyst
8. Reticuloendotheliosis as histiocytosis X (Hand-Schüller-Christian disease)
9. Secondary extension of infectious or neoplastic disease from adjacent sinuses, brain,
skin, bone, nasopharynx, and esophagus
10. Sinus disease including mucoceles

Saunders, 1994:1881-2095.
Margo CE, et al. Psammomatoid ossifying fibroma. Arch Ophthalmol 1986; 107:1347-
1351.
Oh KT, et al. Adenocarcinoma of the esophagus presenting as orbital cellulitis. Arch

Ophthalmol 2000; 118:986-988.
FOSSA FORMATION OF ORBIT (LOCAL EXPANSION OF BONY ORBITAL

WALL CAUSED BY PERSISTENT PRESSURE; BONY CORTEX IS INTACT)
1. Encapsulated benign lacrimal gland tumor

2. Encapsulated malignant lacrimal gland tumor
3. Orbital dermoid
Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WE

Saunders, 1994: 1881-2095.
Jacobs L, et al. Computerized tomography of the orbit and sella turcica. New York:
Raven Press, 1980.
Zizmor J. Orbital radiology in unilateral exophthalmos. In: Turtz AI. Proceedings of the
Centennial Symposium: Manhattan Eye. Ear, and Throat Hospital, Val I. St. Louis: CV
Mosby, 1969.
SUBPERIOSTEAL ORBITAL HEMORRHAGE
1. Generalized diseases with bleeding diatheses

2. Paranasal sinusitis
3. Sudden elevation of cranial venous pressure
4. Trauma
Atalla ML, et al. Nontraumatic subperiosteal orbital hemorrhage. Ophthalmology 2001;
108:183-189.
Hunt KE, Ross JJ. Orbital hemorrhage in the nonoperated eye as a complication of
general endotracheal anesthesia. Arch Ophthalmol 1998; 116L: 105-106.
ORBITAL HEMORRHAGE
1. Idiopathic
2. Other
A. General endotracheal anesthesia
B. Late migration of orbital implant
3. Preexisting vascular tumors
A. Cholesterol granuloma
B. Cystic lymphangiomas
C. Hemorrhagic varix
4. Surgery
A. Retrobulbar injection
B. Rhinoplasty
C. Subtentorial infusion anesthesia
5. Systemic
A. Heparin
B. Thrombolytic
6. Trauma
Chorich LJ, et al. Hemorrhagic ocular complications associated with the use of systemic
thrombolytic agents. Ophthalmology 1998; 105:428-431.
Polito E, et al. Diagnosis and treatment of orbital hemorrhagic lesions. Ann Opthalmol
1994; 26:85-93.
EXPANSION OF ORBITAL MARGINS (USUALLY ASSOCIATED WITH

BENIGN TUMORS OF THE ORBIT)
1. Dermoid
2. Hemangioma
3. Lacrimal gland tumors
4. Meningioma
5. Neurofibroma

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Coleman DJ, et al. Ultrasonography of the eye and orbit. Philadelphia: Lea & Febiger,
1977.
Katz BJ, Nerad JA. Ophthalmic manifestations of fibrous dysplasia. Ophthalmology
1998; 105:2207-2215.
Zizmor J. Orbital radiology in unilateral exophthalmos. In: Turtz AI, ed. Proceedings of
the Centennial Symposium: Manhattan Eye, Ear, and Throat Hospital, Vol I. St. Louis:
CV Mosby, 1969.
HYPERTROPHY OF ORBITAL BONES (HYPEROSTOSIS OR SCLEROSIS OR

BOTH)
1. Acromegaly
2. Anemias of childhood (severe: Cooley, sickle cell, spherocytosis, iron deficiency)
3. Cerebral atrophy (childhood)
4. Craniostenosis
5. Engelmann disease (hereditary diaphyseal dysplasia)
6. Hyperostosis frontalis interna
7. Idiopathic
8. Infantile cortical hyperostosis (Caffey disease)
9. Microcephaly
10. Myotonia atrophic a (myotonic dystrophy, Curschmann-Steinert syndrome)
11. Osteopetrosis (Albers-Schönberg disease)
12. Paget disease (osteitis deformans)
13. Tumors of orbit, including osteoma, fibrous dysplasia (Albright syndrome),
meningioma, metastatic neuroblastoma, mixed tumors of lacrimal gland, transitional cell
carcinomas of the nasopharynx

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Raven Press, 1980.
Teplick JG, Hoskin ME. Roentgenologic diagnosis, 3rd ed. Philadelphia: WB Saunders,
1976.
EXPANSION OF OPTIC CANAL
1. Increased intracranial pressure

2. Inflammatory lesions
A. Chiasmatic arachnoiditis
B. Nonspecific granuloma
C. Sarcoid granuloma
D. Tuberculoma
3. Tumors
A. Meningioma
B. Metastatic sarcoma to choroid
C. Neurofibromatosis (von Recklinghausen syndrome)
D. Optic nerve glioma
E. Retinoblastoma
4. Vascular lesions
A. Arteriovenous malformation
B. Ophthalmic artery aneurysm
Levin LA, Rubin PD. Advances in orbital imaging. Int Ophthalmol Clin 1992; 32:1-25.
Potter GD, Trakel SL. Optic canal. In: Newton TH, Potts DG, eds. Radiology of the skull
and brain, Vol 1, Book 2. St. Louis: CV Mosby, 1971.
the Centennial Symposium: Manhattan Eye, Ear, and Throat Hospital, Vol I. St. Louis:
CV Mosby, 1969.
SMALL OPTIC CANALS
1. Developmental abnormalities
A. Anophthalmos or microphthalmos
B. Enucleation
C. Craniosynostosis (CSO)
2. Dysostoses
A. Osteopetrosis (Albers-Schönberg syndrome)
B. Fibrous dysplasia (Albright syndrome)
C. Pyle disease (craniometaphyseal dysplasia syndrome)
D. Paget disease (osteitis deformans)
3. Inflammatory lesions-osteitis
4. Tumor-meningioma

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Potter GD, Trokel SL. Optic canal. In: Newton TH, Potts DG, eds. Radiology of the skull
and brain, Vol 1, Book 2. S1. Louis: CV Mosby, 1971.
EROSION OF OPTIC CANAL
1. Lateral wall
A. Pituitary tumor
B. Aneurysm of internal carotid artery
C. Craniopharyngioma
D. Tumor of orbital apex
2. Medial wall
A. Carcinoma of sphenoid sinus
B. Mucocele of sphenoid sinus
C. Granuloma of sphenoid sinus
3. Roof
A. Tumor of anterior cranial fossa
B. Surgical unroofing
4. Decrease in length
A. Tumor of orbital apex
5. Complete destruction
A. Malignant tumor
B. Eosinophilic granuloma

Saunders, 1994: 1881-2095.
Potter GD, Trakel SL. Optic canal. In: Newton TH, Potts DG, eds. Radiology of the skull
and brain, Vol 1, Book 2. S1. Louis: CV Mosby, 1971.
the Centennial Symposium: Manhattan Eye, Ear, and Throat Hospital, Vol 1. St. Louis:
CV Mosby, 1969.
ENLARGEMENT OF SUPERIOR ORBITAL FISSURE
1. Carotid cavernous fistula

2. Chronic increased intracranial pressure
3. Extension of infraorbital mass into fissure
4. Intracavernous carotid aneurysm
5. Intracranial chordoma
6. Masses within middle fossa
7. Metastatic carcinoma to sphenoid wings
8. Nasopharyngeal carcinoma-rare
9. Neurofibromatosis including optic nerve glioma
10. Orbital dysplasia
11. Orbital varix
12. Pituitary neoplasm-changes in sella and clinoid process
13. Posterior orbital encephalocele
14. Sarcomas, neurilemoma, or other orbital malignancies

Saunders, 1994:1881-2095.
Grimson BS, Perry DD. Enlargement of the optic disk in childhood optic nerve tumors.
Am J Ophthalmol 1984; 97:627-631.
Shields JA, et al. Orbital neurilemoma with extension through the superior orbital fissure.
Arch Ophthalmol 1986; 104:871-873.
NARROWING OF SUPERIOR ORBITAL FISSURE
1. Chronic hemolytic anemias of childhood

2. Fibrous dysplasia (Albright syndrome)
3. Meningioma
4. Osteitis
5. Osteoblastoma
6. Osteoma
8. Paget disease (osteitis deformans)

Saunders, 1994: 1881- 2095.
Raven Press, 1980.
Kieffer SA. Superior orbital fissure. In: Newton TH, Potts DG, eds. Radiology of the
skull and brain, Vol 1, Book 2. St. Louis: CV Mosby, 1971.
SMALL ORBIT
1. Anophthalmos
2. Enucleation
3. Microphthalmos
4. Mucocele
Kieffer SA. Orbit. In: Newton TH, Potts DG, eds. Radiology of the skull and brain, Vol
1, Book 2. St. Louis: CV Mosby, 1971.
Sarnat BG. Eye and orbital size in the young and adult. Ophthalmologica 1982; 185:74-
89.
LARGE ORBIT
1. Congenital
A. Dysplasia
B. Glaucoma
C. Serous cysts
2. Pseudotumor
3. Tumors within the muscle cone
A. Hemangiomas
B. Neurofibroma
C. Optic glioma
D. Orbital varix
E. Retinoblastoma
Saunders, 1994: 1881-2095.
Kieffer SA. Orbit. In: Newton TH, Potts DG, eds. Radiology of the skull and brain, Vol
1, Book 2. St. Louis: CV Mosby, 1971.
HEMATIC ORBITAL CYSTS (BLOOD CYST OF ORBIT)
1. Blood dyscrasia
2. Cavernous hemangioma
3. Childbirth
4. Lymphangioma
5. Orbital blunt trauma
6. Spontaneous hemorrhage
7. Vascular disease

Saunders, 1994:1881-2095.
Jacobson DM, et al. Maternal orbital hematoma associated with labor. Am J Ophthalmol
1988; 105:547-553.
Shapiro A, et al. A clinicopathologic study of hematic cysts of the orbit. Am J

Ophthalmol 1986; 102:237-241.
2
Lids
CONTENTS
Mongoloid palpebral fissure 46
Antimongoloid palpebral fissure 47
Pseudoptosis 48
Blepharoptosis 49
Syndromes and diseases associated with ptosis 52
Ptosis-Diagnostic table 55
Specific blepharoptosis 57
Horner syndrome 59
Horner syndrome-Diagnostic table 61
Ptosis of lower lid 62
Lagophthalmos 62
Pseudo-lid retraction 62
Lid retraction 62
Lid lag 64
Blepharospasm 65
Facial palsy 66
Infrequent blinking 68
Frequent blinking 68
Lid edema 69
Bleeding of the eyelid 77
Ectropion 78
Entropion 79
Epicanthus 80
Hypopigmentation 81
Hyperpigmentation 83
Tumors of eyelids 86
Tumors of eyelids-Diagnostic table 87
Xanthelasma 88
Chronic blepharitis 89
Acute blepharitis 94
Thickened eyelids 97
Blepharophimosis 97
Euryblepharon 98
Lid coloboma 98
Necrosis of eyelids 99
Poliosis 99
Trichomegaly 99
Madarosis 00
Madarosis-Diagnostic table 102
Distichiasis 104
Coarse eyebrows 105
Synophrys 105
Hertogh sign 105
Lid myokymia 105
Preseptal cellulitis of eyelid 106
Telecanthus 106
Ankyloblepharon 107
Flaring of nasal part of eyebrow 107
High arched brow 108
Absent brow hair 108
Trichiasis 108
MONGOLOID PALPEBRAL FISSURE (TEMPORAL CANTHUS HIGHER

THAN NASAL CANTHUS)
1. Esotropia syndrome
2. Exotropia syndrome
3. Amniogenic band syndrome (Streeter dysplasia)
4. Anhidrotic ectodermal dysplasia
5. Cebocephalia (fetalis hypoplastica)
6. Chondrodystrophia (Conradi syndrome)
8. Congenital spherocytic anemia
9. Crouzon syndrome (hereditary craniofacial dysostosis)
10. Duplication 14Q syndrome
11. Femoral-facial syndrome
12. Fetal hydantoin syndrome
13. Hereditary ectodermal dysplasia syndrome (Siemen syndrome)
15. Jarcho-Levin syndrome
16. Klinefelter XXY syndrome (gynecomastia-aspermatogenesis syndrome)
17. Laurence-Moon-Biedl syndrome (retinitis pigmentosa-polydactyl-adiposogenital
syndrome)
18. Meckel syndrome (dysencephalia-splanchnocystic syndrome)
19. Miller-Dieker syndrome
*20. Mongoloid (trisomy or Down syndrome)
*21. Asian persons
22. Peters trisomy 5p (Peters anomaly)
23. Otopalataodigital syndrome
25. Pleonosteosis syndrome (Leri syndrome, carpal tunnel syndrome)
26. Prader-Willi syndrome
27. Rhizomelic chondrodysplasia punctata syndrome
28. Trisomy mosaic and 9p- syndromes
30. Trisomy 6Q syndrome
31. Trisomy 9Q syndrome
32. Trisomy and 18q syndrome
34. XXXXX syndrome
35. XXXXY syndrome
36. 4p- syndrome
37. 5p- syndrome
Isenberg SJ. The eye in infancy. Chicago: Year Book Medical, 1989.
ANTIMONGOLOID PALPEBRAL FISSURE (DOWNWARD DISPLACEMENT

OF TEMPORAL CANTHUS)

2. Acrocephalosyndactylia (Apert syndrome)
3. Esotropia and exotropia
4. Bird-headed dwarf syndrome (Seckel syndrome)
5. Baraitser-Winter syndrome
6. Cardiofaciocutaneous syndrome
7. Cerebral gigantism (Sotos syndrome)
8. Cleft palate
10. Cloverleaf cranium
11. Coffin-Lowry syndrome
12. Cohen syndrome
13. Congenital facial hemiatrophy (Möbius syndrome)
14. Craniocarpotarsal dysplasia (Freeman-Sheldon syndrome; whistling face syndrome)
15. Craniofacial dysostosis (Crouzon syndrome)
17. De Lange syndrome (congenital muscular hypertrophy -cerebral syndrome)
18. Di George syndrome
19. Epidermal nevus syndrome (ichthyosis hystrix)
20. Lethal multiple pterygium syndrome (LMPS)
21. Linear nevus sebaceous of Jadassohn (Jadassohn-type anetoderma)
22. Mandibulofacial dysostosis (Franceschetti syndrome and Treacher-Collins syndrome)
23. Marchesani syndrome (dystrophia mesodermalis congenita hyperplastica)
24. Maxillofacial dysostosis
25. Nager syndrome
27. Obesity-cerebral-ocular-skeletal anomalies syndrome
28. Oculoauriculovertebral dysplasia (Goldenhar syndrome)
29. Oculomandibulofacial dyscephaly (Hallermann-Streiff syndrome)
30. Organoid nevus syndrome
31. Otopalatodigital syndrome (OPD)
32. Pyknodysostosis
33. Partial trisomy of long arm of chromosome 630. Pseudo-Ullrich-Turner syndrome
34. Ring D chromosome
35. Rubinstein-Taybi syndrome (broad thumbs syndrome)
36. Ruvalcaba syndrome
37. Saethre-Chotzen syndrome
38. 3-P syndrome
*39. Trauma
43. Trisomy 20P syndrome
44. Trisomy syndrome (E syndrome)
46. Wolf syndrome (chromosome partial deletion syndrome)
47. 4q- syndrome
48. 21q syndrome
49. 9p syndrome
PSEUDOPTOSIS
Pseudoptosis includes conditions that simulate ptosis, but lid droop is not the result of
levator malfunction, and ptosis is usually corrected when the causative factors are cleared
up or removed.
1. Due to globe displacement

A. Anophthalmia including poorly fitting prosthesis
*B. Enophthalmos such as that resulting from blowout fracture of the floor of the
orbit or atrophy of orbital fat
*C. Microphthalmia
*D. Phthisis bulbi
E. Hypotony and inward collapse of eye
F. Cornea plana
G. Hypotropia of that eye or hypertropia of the other eye
2. Due to mechanical displacement of the lid
A. Inflammation
(1) Trachoma-thick, heavy lid
*(2) Chalazion or hordeolum
(3) Elephantiasis
(4) Chronic conjunctivitis-conjunctival thickening
(5) Traumatic or infectious edema involving the lid
*(6) Blepharitis
(7) Corneal foreign body
(8) Contact lens
(9) Sinusitis, cellulitis
B. Tumors, especially fibromas, lipomas, or hemangiomas
C. Scar tissue due to burns, physical trauma, and lacerations that can bind the lid
down
D. Tumors of lacrimal gland-S-shaped lid
*3. Dermatochalasis (ptosis adiposa, baggy lids, "puffs" -senile atrophy of the lid skin)
4. Blepharochalasis-a rare condition occurring in young persons, characterized by
recurrent bouts of inflammatory lid edema with subsequent stretching of the skin
5. The oriental lid-the palpebral fissure is narrower than normal and the upper lid rarely
has a furrow; hence, the fold usually hangs down to or over the lid margin.
6. Dissociated vertical deviation (DVD)
7. Duane syndrome (retraction syndrome)
8. Blepharospasm-eyebrow lower than normal, hemifacial spasm
9. Contralateral widening of the lid fissure as pseudoproptosis (see p. 4), exophthalmos,
or lid retraction (see p. 62)
10. Vertical strabismus
Beard C. Ptosis. St. Louis: CV Mosby, 1969.
Beyer CK, et al. Naso-orbital fractures, complications, and treatment. Ophthalmology

1982; 89:456.
Crawford JS. Ptosis: is it correctable and how? Ann Ophthalmol 1971; 3:452-456.
Huber A. Eye symptoms in brain tumors, 2nd ed. St. Louis: CV Mosby, 1971.
BLEPHAROPTOSIS (PTOSIS, DROOPY UPPER LID; WEAK LEVATOR

PALPEBRAE SUPERIORIS MUSCLE)
1. Congenital ptosis
*A. Simple (most congenital ptosis)-may be the result of autosomal dominant
inheritance
B. Complicated ptosis
(1) Ptosis with ophthalmoplegia (most congenital ptosis)-the most
commonly involved muscle is the superior rectus
(2) Ptosis with other lid deformities such as epicanthus, blepharophimosis,
microphthalmia, and lid coloboma-may be hereditary
(3) Synkinetic (paradoxical) ptosis-aberrant nervous connections from the
other extrinsic muscles of the eye and jaw to the levator muscle
a. Marcus Gunn phenomenon (jaw-winking reflex)-motor root of
the fifth cranial nerve to the muscle of mastication also is
misdirected through the third nerve to the levator muscle.
b. Phenomenon of Marin Amat (reverse jaw-winking reflex)
c. Misdirected third nerve syndrome-bizarre eyelid movements that
may accompany various eye movements; the ptotic eyelid may rise
as the medial rectus, the inferior rectus, or the superior rectus
muscle contracts.
(4) Homer syndrome
C. Involutional ptosis
D. Mechanical
(1) Periorbital tumor
(2) Neuroma, neurofibroma
(3) Cicatricial skin changes
2. Acquired ptosis
A. Traumatic ptosis
(1) Eyelid laceration
(2) Postsurgical ptosis
a. Anterior transposition of inferior oblique muscle
b. Enucleation
c. Orbital operation
d. Cataract operation
e. Radial keratotomy
(3) Foreign bodies lying in the roof of the orbit
(4) Fracture of orbital roof, also following contusion with resulting
hematoma but without fracture
(5) Air-blast injury
(6) Botulinum toxin treatment of strabismus and blepharospasm
(7) Prolonged hard contact lens wear
(8) Infratemporal fossa foreign body
B. Neurogenic ptosis
(1) Peripheral involvement of the third nerve
(2) Basilar, cortical, and nuclear lesions
(3) Cerebral hemorrhages, tumors, or abscesses
(4) Multiple neuritis, nerve syphilis, or multiple sclerosis
(5) Homer syndrome-lower lid higher than other lower lid
(6) Familial dysautonomia (Riley-Day syndrome)
(7) Misdirected third nerve syndrome-following third nerve palsy the
fibers do not regrow into their respective muscles.
(8) Aseptic meningitis, transient
(9) Pituitary tumor
C. Myogenic ptosis
(1) Primary muscular atrophy (late familial ptosis); ptosis is usually the
only symptom.
(2) Dystrophia myotonia, in which there is dystrophia not only of the
extraocular muscles but also of the face, neck, and extremities
(3) Myasthenia gravis, nonfamilial acquired ptosis
(4) The congenital fibrosis syndrome characterized by bilateral ptosis and
gradual fibrosis of all the extraocular muscles
(5) Oculopharyngeal muscular dystrophy characterized by dysphagia and
progressive bilateral ptosis
(6) Progressive familial myopathic ptosis and involvement of one, some,
or all extraocular (and no other) muscles of one or both eyes
(7) Late spontaneous unilateral ptosis
(8) Amyloid degeneration with involvement of the levator muscle
*(9) Senility-loss of general muscle tone and atrophy of orbital fat
(10) Ptosis and normal pregnancy
(11) Hyperthyroidism and ptosis-following active stages
(12) Drugs, including the following:
adenine arabinoside diphtheria and tetanus nalidixic acid(?)
adrenal cortex injection toxoids and pertussis nialamide
alcohol (DPT) opium
aldosterone disulfiram(?) oral contraceptives
allobarbital fludrocortisone paramethasone
amobarbital fluorometholone pentobarbital
amodiaquine flu-prednisolone phencyclidine
aprobarbital F3T phenelzine
aurothioglucose gold Au-198 phenobarbital
aurothioglycanide gold sodium thiomalate phenoxybenzamine
barbital heptabarbital prednisolone
betamethasone hexethal prednisone
butabarbital hexobarbital primidone
butalbital hydrocortisone probarbital
butallylonal hydroxychloroquine secobarbital
butethal idoxuridine succinylcholine
carbon dioxide isocarboxazid sulthiame
carbromal isosorbide dinitrate(?) talbutal
chloral hydrate loxapine tetraethylammonium
chloroquine measles virus vaccine thiamylal
cocaine(?) (live) thiopental
cortisone medrysone tolazoline
cyclobarbital mephenesin tranylcypromine
cyclopentyl mephobarbital triamcinolone
allylbarbituric acid metharbital trichloroethylene
cyclopentobarbital methitural trifluorothymidine
desoxycorticosterone methohexital tubocurarine
dexamethasone methyl alcohol vidarabine
dextrothyroxine methylpentynol vinbarbital
digitalis methylprednisolone vinblastine
dimethyl tubocurarine metocurine iodide vincristine
(13) Corticosteroid ptosis-prolonged use of topical corticosteroid therapy
(14) Mascara ptosis-due to subconjunctival deposits of mascara
(15) Ptosis associated with chronic conjunctivitis and uveitis
(16) Use of botulinum toxin
D. Protective ptosis following injury to the eye
E. Mechanical ptosis
(1) Tumor
a. Benign tumor-such as neurofibroma or hemangioma
b. Malignant tumor-such as basal cell carcinoma, squamous cell
carcinoma, malignant melanoma, or rhabdomyosarcoma
c. Metastatic lesion-such as from breast or lung
d. Sinus extension-such as mucocele of frontal sinus
(2) Blepharochalasis-hereditary with recurrent attacks of severe edema and
residual damage to the tissues
(3) Cicatricial ptosis-such as that secondary to cicatricial conjunctivitis
(see p. 49-50) or surgical trauma to the superior fornix
(4) Contact lens migration
(5) Palpebral form of vernal conjunctivitis
(6) Intracranial extension-such as chordoma
Bodker FS, et al. Acquired blepharoptosis secondary to essential blepharospasm.

Ophthalmic Surgery 1993; 24: 546-550.
Frueh BR. The mechanistic classification of ptosis. Ophthalmology 1980; 87:1019.
Grant CA, et al. An infratemporal fossa foreign body presents as an infraorbital mass.
Arch Ophthalmol 2000; 118: 993-995.
Hertle RW, et al. Congenital unilateral fibrosis, blepharoptosis, and enophthalmos

syndrome. Ophthalmology 1992; 99:347-355.
Kushner BJ. The effect of anterior transposition of the inferior oblique muscle on the
palpebral fissure. Arch OphthalmoI 12ooo; 18:1542-1544.
Vaughn GL, et al. Variable diplopia and blepharoptosis after orbital floor fracture repair.
Am J Ophthalmol 1994; 117:407-409.
SYNDROMES AND DISEASES ASSOCIATED WITH PTOSIS
1. Aarskog syndrome (faciogenital dysplasia)-x-linked

2. Acquired immunodeficiency syndrome
3. Addison disease (idiopathic hypoparathyroidism)
4. Alacrima congenital with distichiasis, conjunctivitis, keratitis-autosomal dominant
5. Albers-Schönberg syndrome (marble bone disease)
6. Albright syndrome (osteitis fibrosa disseminata)
8. Apert syndrome (acrocephalosyndactylia syndrome)
9. Arteriovenous fistula
10. Axenfeld-Schurenberg syndrome (cyclic oculomotor paralysis)
11. Babinski-Nageotte syndrome (medullary tegmental paralysis)
12. Basal cell carcinoma
13. Bassen-Kornzweig syndrome (abetalipoproteinemia)
14. Bell palsy (idiopathic facial paralysis)
15. Bing-Neel syndrome (Bing disease)
16. Blepharophimosis syndrome
17. Bonnet-Dechaume-Blanc syndrome (neuroretinoangiomatosis)
18. Bonnevie- Ullrich syndrome (pterygolymphangiectasia)
19. Botulism
20. Brown syndrome (superior oblique tendon sheath syndrome)
22. Cavernous sinus syndrome (Foix syndrome)
23. Cerebral palsy
24. Cestan-Chenais syndrome (Cestan syndrome)
25. Chromosome long-arm deletion syndrome
26. Chromosome partial deletion (long-arm) syndrome (de Grouchy syndrome)
27. Chromosome short-arm deletion syndrome
28. Congenital fibrosis syndrome (all extraocular muscles)
29. Congenital fibrosis of the inferior rectus with ptosis-autosomal dominant
*30. Congenital ptosis
A. Simple failure of peripheral differentiation of muscles-dominant
B. Ptosis with blepharophimosis-dominant
C. Ptosis due to ophthalmoplegia-autosomal dominant
31. Craniocarpotarsal dysplasia (Freeman -Sheldon syndrome)
32. Craniocervical syndrome (whiplash injury)
33. Cretinism (juvenile hypothyroidism)
34. Creutzfeldt-Jakob syndrome (spastic pseudosclerosis)
36. Crouzon syndrome (craniofacial dysostosis)
37. Cushing syndrome (2) (cerebellopontine angle syndrome)
38. Dandy-Walker syndrome (atresia of foramen Magendie)
39. Dawson disease (subacute sclerosing panencephalitis)
40. Dejerine-Klumpke syndrome (lower radicular syndrome)
42. Devic syndrome (ophthalmoencephalomyelopathy)
43. Diphtheria
44. Dubowitz syndrome (dwarfism-eczema-peculiar facies)
45. Duck-bill lips, low-set ears-autosomal dominant
46. Eaton-Lambert syndrome (myasthenic syndrome)
47. Eclampsia and preeclampsia
48. Ehlers-Danlos syndrome (fibrodysplasia elastic generalisata)
49. Engelmann syndrome (osteopathia hyperostotica scleroticans multiplex infantalis)
51. Erb-Goldflam syndrome (myasthenia gravis)
52. Erysipelas (St. Anthony fire)
53. Fabry syndrome
54. Faciorenal acromesometic syndrome
55. Fisher syndrome (ophthalmoplegia-ataxia-areflexia syndrome)
57. Fetal trimethadione
58. Foramen lacerum syndrome (aneurysm of internal carotid artery syndrome)
59. Freeman-Sheldon syndrome
60. Garcin syndrome (half-base syndrome)
61. Gerlier disease (paralytic vertigo)
62. Gillum-Anderson syndrome (dominant blepharoptosis, high myopia)
63. Guillain-Barré syndrome (acute infectious neuritis)
64. Hairy elbow syndrome
65. Hemangiomas
66. Herpes zoster
67. Hodgkin disease
68. Horner syndrome (cervical sympathetic paralysis)
69. Hunter syndrome [mucopolysaccharidosis (MPS) II]
70. Hurler disease (MPS I)
71. Hyperammonemia
73. Hyperthyroidism (Basedow syndrome)
74. Hypocalcemia
*75. Hypoparathyroidism
76. Hysteria
77. Infectious mononucleosis
78. Influenza
79. Jugular foramen syndrome (Vernet syndrome)
80. Kearns-Same syndrome
81. Kiloh-Nevin syndrome (muscular dystrophy of external ocular muscles)
82. Kohn-Romano syndrome (blepharoptosis, blepharophimosis, epicanthus inversus,
telecanthus)
83. Komoto syndrome (congenital eyelid tetrad)
84. Krause syndrome (congenital encephaloophthalmic dysplasia)
85. Kugelberg-Welander syndrome (progressive proximal muscle atrophy)
86. Kussmaul disease (necrotizing angiitis)
87. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-
adiposogenital syndrome)
88. Leigh disease (subacute necrotizing encephalomyelopathy)
90. Lymphangioma
91. Lymphedema
92. Malaria
93. Malignant hyperthermia syndrome
94. Maple-syrup urine disease (branched-chain ketoaciduria)
95. Marcus Gunn syndrome (jaw-winking syndrome)
96. Marin Amat syndrome (inverted Marcus Gunn syndrome)
97. MERRF syndrome
98. Micro syndrome
99. Misdirected third nerve syndrome
100. Möbius syndrome (congenital paralysis of the sixth and seventh nerves)
101. Morquio syndrome (keratosulfaturia)
102. Mucormycosis
103. Multiple sclerosis (disseminated sclerosis)
104. Myopathy, centronuclear with external ophthalmoplegia-autosomal dominant
105. Myotonic dystrophy syndrome (Curschmann-Steinert syndrome)
106. Myotubular myopathy-autosomal recessive or x-linked
107. Naffziger syndrome (scalenus anticus syndrome)
108. Neurilemoma
109. Neuroblastoma
110. Neurofibromatosis
111. Nonne-Milroy-Meige disease (congenital trophedema)
113. Oculopharyngeal muscular dystrophy
114. Ophthalmoplegic migraine syndrome
115. Ophthalmoplegic-retinal degeneration (Kearns-Sayre syndrome)
116. Orodigital-facial syndrome (Papillon-Léage and Psaume syndrome)
117. Pachydermoperiostosis (Touraine-Solente-Gole syndrome)
118. Pancoast syndrome (superior pulmonary sulcus syndrome)
119. Parinaud syndrome (paralysis of vertical movements)
120. Parkinson syndrome (paralysis agitans)
121. Parry-Romberg syndrome (progressive facial hemiatrophy)
123. Pierre-Robin syndrome (micrognathia-glossoptosis syndrome)
124. Poliomyelitis
125. Progressive intracranial arterial occlusion syndrome
126, Purpura and ptosis-combined inheritance with male-to-male transmission
127. Raeder syndrome (paratrigeminal paralysis)
128. Retraction syndrome (Duane syndrome)-autosomal dominant
129. Retroparotid space syndrome
130. Riley-Day syndrome (congenital familial dysautonomia)
134. Scleroderma (progressive systemic sclerosis)
135. Scurvy (vitamin C deficiency)
136. Shy-Gonatas syndrome (similar to Hunter and Refsum syndrome)
137. Smith-Lemli-Opitz syndrome (cerebrohepatorenal syndrome)
138. Smith syndrome (facioskeletogenital dysplasia)
139. Sparganosis
140. Spider bites
141. Strabismus and ectopic pupils-autosomal dominant
142. Subclavian steal syndrome
143. Syphilis (acquired lues)
144. Syringomyelia (Passow syndrome)
145. Temporal arteritis syndrome (Hutchinson-Horton-Magath-Brown syndrome)
146. Thirteen Q syndrome (microcephaly, high nasal bridge, thumb hypoplasia)
147. Tolosa-Hunt syndrome (painful ophthalmoplegia)
148. 3p syndrome
149. Trachoma
150. Treft syndrome (optic atrophy and hearing loss)
151. Triploidy (chromosomes instead of 46)
152. Trisomy (E syndrome)
153. Tuberculosis
154. Tunbridge-Paley disease (juvenile diabetes, optic atrophy and hearing loss)
156. van Bogaert-Hozay syndrome
157. Vertebral fusion, posterior lumbosacral with ptosis-autosomal dominant
158. von Herrenschwand syndrome (sympathetic heterochromia)
159. von Recklinghausen syndrome (neurofibromatosis)
161. Wallenberg syndrome (dorsolateral medullary syndrome)
162. Weber syndrome (cerebellar peduncle syndrome)
163. Wernicke syndrome (hemorrhagic polioencephalitis superior syndrome)
Larned DC, et al. The association of congenital ptosis and congenital heart disease.
Ophthalmology 1986; 93:492-494.
McKusick VA. Mendelian inheritance in man, 12th ed. Baltimore: Johns Hopkins
University Press, 1998.
Extracted Table Ptosis
SPECIFIC BLEPHAROPTOSIS
1. Unilateral ptosis with dilated pupil-tumor or abscess of temporal lobe and third nerve
palsy
2. Unilateral ptosis with miosis-midbrain lesion near the posterior commissure and
Horner syndrome
3. Ptosis with disturbance of integrated ocular movement-lesion near superior colliculus
4. Bilateral ptosis with small immobile pupils and loss of upward rotation of eyeballs-
lesion near posterior commissure
5. Ptosis with loss of voluntary elevation but normal involuntary elevation of the lid when
the eye looks up-supranuclear lesion
6. Ptosis in repose and normal elevation with active motion-hereditary cerebellar ataxia
of Pierre-Marie
7. Ptosis onset in adolescent-familial chronic external ophthalmoplegia
8. Ptosis may be early and only sign of nuclear paralysis in:
A. Botulism
B. Multiple sclerosis (disseminated sclerosis)
C. Hemorrhagic superior poliomyelitis of Wernicke
D. Tabes
E. Vasospasm of ophthalmoplegic migraine
9. Ptosis with cranial nerve dysfunction suggests a basal lesion, such as the following:
A. Aneurysm
B. Epidemic paralyzed vertigo (Gerlier disease)
*C. Herpes zoster
D. Meningitis
E. Polyneuritis of cranial nerves
*F. Trauma
10. Transient ptosis
A. Acute exanthema
B. Acute infection such as erysipelas
*C. Botulinum toxin injection
D. Eclampsia
E. Exogenous poisons such as those due to alcohol, lead, carbon monoxide,
arsenic, snake venom
F. Hematoma
G. Influenza
H. Scurvy (vitamin C deficiency)
11. Ptosis with orbicularis weakness-muscle disease
12. S-shaped ptosis
A. Chronic chalazion
B. Cyst on lateral border of tarsus
C. Dermoid
D. Floppy eyelid syndrome
E. Lacrimal gland enlargement or prolapse
F. Lateral levator palpebrae superioris muscle dehiscence
G. Neurofibromatosis
H. Trachoma
I. Drugs, including the following:
Dunn WJ, et al. Botulinum toxin for the treatment of dysthyroid ocular myopathy.
Ophthalmology 1986; 93: 4770-4775.
Gerner EW, Hughes SM. Floppy eyelid with hyperglycinemia. Am J Ophthalmol 1984;
98:614-615.
Haessler FH. Eye signs in general disease. Springfield, IL: Charles C Thomas, 1960.
HORNER SYNDROME
Homer syndrome comprises paralysis of sympathetic nerve supply with lid ptosis,
miosis, apparent enophthalmos, frequently dilatation of the vessels with absence of
sweating (anhidrosis) on homolateral side; the pupil demonstrates a decreased sensitivity
to cocaine and hypersensitivity to adrenalin and may have heterochromia with congenital
Homer syndrome.
1. Region of first neuron-lesions of hypothalamus and diencephalic region also suggest

diabetes insipidus, disturbed temperature regulation, adiposogenital syndrome, and
autonomic epidemic epilepsy of Penfield.
A. Arnold-Chiari malformation
B. Basal meningitis, such as in syphilis
C. Base-of-skull tumors (e.g., melanoma)
D. Multiple sclerosis
E. Pituitary tumor
F. Tumor of the third ventricle
G. Midbrain, such as in syphilis
H. Pons, such as in intrapontine hemorrhage
I. Medulla, such as in Wallenberg syndrome (lateral medullary syndrome)-
thrombosis of posterior inferior cerebellar artery
J. Cervical region
(1) Syringomyelia
(2) Tumor
(3) Injury as traumatic dislocation of cervical vertebrae or dissection of the
vertebral artery
(4) Syphilis (acquired lues)
(5) Poliomyelitis
(6) Meningitis
(7) Amyotrophic lateral sclerosis
(8) Related to scleroderma and facial hemiatrophy
(9) Vascular malformation such as agenesis of internal carotid artery
2. Region of second neuron
A. Spinal birth injury-Klumpke paralysis with injured lower brachial plexus
B. Cervical rib
C. Charcot-Tobias syndrome
D. Thoracic lesions
(1) Pancoast tumor-in apex of lung, such as carcinoma or tuberculosis
(2) Aneurysm of aorta, subclavian, or carotid artery
(3) Central venous catheterization
(4) Mediastinal tumors
(5) Lymphadenopathy of Hodgkin disease, leukemia, lymphosarcoma, or
tuberculosis
(6) Stellate ganglion block
(7) Tube thoracostomy
E. Neck
*(1) Enlarged lymph gland, tumors, aneurysm, and thyroid gland
(2) Carcinoma of esophagus
(3) Retropharyngeal tumors
(4) Neuroma of sympathetic chain
(5) Intraoral trauma with damage to internal carotid plexus
(6) Thin intervertebral foramina of spinal cord, such as in
pachymeningitis, hypertrophic spinal arthritis, ruptured intervertebral disc,
and meningeal tumors
(7) Traction of sternocleidomastoid muscle, such as from positioning on
operating table
(8) Complications of tonsillectomy
(9) Mandibular tooth abscess
*(10) Lesions of middle ear, such as in acute purulent otitis media and
petromastoid operation
(11) Carotid artery dissection
(12) Internal carotid artery occlusion
3. Region of third neuron
A. Aneurysm of internal carotid and its branches
B. Paratrigeminal syndrome (Raeder syndrome)
C. Cavernous sinus syndrome (Foix syndrome)
D. Tumors of cysts of orbit
E. Drugs can affect any region and include the following:
acetophenazine levodopa promethazine
alseroxylon lidocaine propiomazine
bupivacaine mepivacaine propoxycaine
butaperazine mesoridazine rauwolfia serpentina
carphenazine methdilazine rescinnamine
chloroprocaine methotrimeprazine reserpine
chlorpromazine oral contraceptives syrosingopine
deserpidine perazine thiethylperazine
diacetylmorphine pericyazine thiopropazate
diethazine perphenazine thioproperazine
ethopropazine piperacetazine thioridazine
etidocaine prilocaine trifluoperazine
fluphenazine procaine triflupromazine
guanethidine prochlorperazine trimeprazine
influenza virus vaccine promazine
F. Cluster headaches (migrainous neuralgia)

G. Herpes zoster
H. Migraine
I. Fetal varicella syndrome
Ryan FH, et al. Congenital Horner's syndrome resulting from agenesis of the internal
carotid artery. Ophthalmology 2000; 107: 185-188.
Smith EF, et al. Herpes zoster ophthalmicus as a cause of Horner syndrome. J Clin
Neuro-Ophthalmol 1993; 13: 250-253.
Extracted Table Horner Syndrome
PTOSIS OF LOWER LID (UNCOMMON DROOPING OF LOWER LID SO

THAT LID MARGIN IS ADJACENT TO GLOBE BUT BELOW LIMBUS)
1. Blepharophimosis syndrome
*2. Cicatricial with mechanical displacement by scar, tumor, or skin disease; may be
associated with ectropion
*3. Paralytic due to lower lid lagophthalmos
4. Pseudoptosis such as in exophthalmos and higher degrees of myopia
5. Idiopathic
Fox SA. Idiopathic blepharoptosis of lower eyelid. Am J Ophthalmol 1972; 74:330-331.
Leatherbarron B, Collin JR. Eyelid surgery in facial palsy. Eye 1991; 5:585-590.
LAGOPHTHALMOS (INABILITY TO CLOSE EYELIDS VOLUNTARILY)
*1. Physiologic-many people sleep with their eyes open, especially Asian people
2. Orbital-extreme proptosis
3. Mechanical-scarring of the lids or retractor muscles
4. Paralytic
A. Seventh nerve palsy (see p. 66)
B. Leprosy
C. Lesions of cerebral cortex and its projections, including bilateral frontal lesions
5. Psychological
A. Failure to comprehend the command
*B. Unwillingness to comply with the command
Harvey JT, Anderson RL. Lid lag and lagophthalmos. Ophthalmol Surg 1981; 12:338.
Lessell S. Supranuclear paralysis of voluntary lid closure. Arch Ophthalmol 1972;

88:241-244.
PSEUDO-LID RETRACTION
*1. Exophthalmos
2. Unilateral high axial myopia
3. Unilateral congenital glaucoma
4. Congenital cystic eyeball
5. Abnormalities of orbit
A. Asymmetry
B. Shallow such as in Crouzon disease (dysostosis craniofacialis)
C. Harlequin-shallow orbit with arched superior and lateral wall, such as in
hypophosphatasia
6. Ptosis of other eyelid
Fox SA. The palpebral fissure. Am J Ophthalmol 1966; 62:73-78.
Walsh FB, Hoyt WF. Clinical neuro-ophthalmology, 4th ed. Baltimore: Williams &
Wilkins, 1985.
LID RETRACTION
Lid retraction is defined normally as more than 85% of vertical palpebral fissures
and 10 mm or less with the eyelids just concealing the corneoscleral limbus at the 12 and
6 o' clock meridians.
1. Lid retraction with upward movement of eye

A. Congestive dysthyroid disease
B. Deficiency in upward gaze-following rectus operation or weakness of superior
rectus
C. Excessive stimulation of levator muscles in Bell phenomenon with seventh
nerve palsy
D. Levator muscles receive excessive stimuli from nerve fiber of superior rectus
E. Pretectal or periaqueductal lesion in midbrain
2. Lid retraction with downward movement of eye
A. Aberrant regeneration of third nerve of inferior rectus to levator (pseudo-
Graefe phenomenon) - elevation of lid in downward gaze
B. Brown syndrome (superior oblique tendon sheath syndrome)
C. Extrapyramidal syndrome of postencephalic parkinsonism and progressive
supranuclear palsy
D. Failure of levator to relax on downward movement of eye
(1) Secondary neuromuscular
*(2) Mechanical, such as from a scar
E. Noncongestive type of dysthyroid exophthalmos (Graefe sign)-lid lag in
downward gaze
3. Lid retraction with horizontal gaze
A. Duane syndrome (retraction syndrome)
B. Underaction of lateral rectus muscle and spillover to levator causing widening
4. Lid retraction because of supranuclear lesions-usually bilateral when due to lesion in or
about posterior commissure (Collier sign, tucked lids, posterior fossa stare)
A. Bulbar poliomyelitis
B. Chorea (Huntington hereditary chorea)
C. Closed head injury associated with defective adduction of eyes, coarse
nystagmus, nuclear palsy, pyramidal signs
D. Coma due to disease of ventral midbrain and pons
E. Craniostenosis
F. Epidemic encephalitis
G. Hydrocephalic infants
H. Hydrophobia
I. Hysteria
J. Malingering
K. Meningitis
L. Multiple sclerosis (disseminated sclerosis)
M. Parinaud syndrome (divergence paralysis)
N. Parkinson disease (paralysis agitans)
O. Russell syndrome
P. Sylvian aqueduct syndrome (Koerber-Solus-Elschnig syndrome)
Q. Syphilis (tabes)
R. Tumors of the midbrain; meningiomas of sphenoid wing; sellar, parasellar, and
suprasellar tumors; and frontal or temporal lobe tumors
S. von Economo syndrome (encephalitis lethargic a)
5. Lid retraction because of neuromuscular disease-commonly asymmetric or unilateral
A. Drugs
(1) Phenylephrine and other sympathomimetics
(2) Prostigmin and Tensilon, especially with myasthenic levator
involvement
(3) Succinylcholine, subparalytic doses
(4) Thyroid extract
B. Fuch phenomenon-healing of injured third nerve, previously ptotic lid has
involuntarily spastic raising with movements of eyes
C. Infant lid retraction-transient because of maternal hyperthyroidism
D. Irritation of cervical sympathetic nerve (Homer syndrome)
*E. Mechanical suspension of lid such as that due to scar, tumor, surgical
attachment to frontalis muscle, or shortening of levator muscle or following
glaucoma filtering procedures
F. Peripheral seventh nerve paresis with loss of orbicularis oculi muscle tone
6. Lid retraction with myopathic disease
A. Associated with hepatic cirrhosis
B. Thyroid myopathy (Graves disease, Basedow syndrome)
(1) Dalrymple sign-widening of palpebral fissure
(2) Stellwag sign-retraction of upper lid associated with infrequent or
incomplete blinking
7. Lid retraction following operations on vertical muscles, such as recession of superior
rectus muscle or simultaneous recession and restriction of the levator by common fascial
check ligament between the two muscles
8. Paradoxical lid retraction because of paradoxical levator innervation
A. Defective ocular abduction with abducens palsy
B. Lid retraction associated with ptosis of the opposite eyelid (levator denervation
supersensitivity)
C. Misdirection of third nerve axons (following acquired or congenital lesions)-
occurs on attempt to adduct, elevate, or depress eye
D. Movement of lower jaw
(1) Contraction of external pterygoid muscle by opening mouth (Marcus
Gunn)
(2) Contraction of internal pterygoid muscle by closing the mouth
9. Physiologic
A. Act of surprise
B. Slow onset of blindness, such as that secondary to glaucoma and optic atrophy
C. Time of attention
Collins JR, et al. Congenital eyelid retraction. Br J Ophthalmol 1990; 74:542-544.
Dixon R. The surgical management of thyroid-related upper eyelid retraction.

Ophthalmology 1982; 89:52.
Wilkins, 1985.
LID LAG
Lig lag is defined as occurring when the patient looks down and the eyelids lag behind
briefly.
1. Congenital-usually in association with congenital ptosis

2. Hepatic failure
3. Iatrogenic-following ptosis surgery
4. Mechanical-scars of the upper lid
5. Myopathic disease
*A. Graefe sign-thyroid myopathy-the upper lid pauses and then follows the eye
downward (Basedow syndrome)
B. Myotonic dystrophia
C. Periodic myotonic lid lag-familial (hyperkalemic) myotonic periodic paralysis
6. Neuromuscular disease
A. Excessive intake of thyroid extract
B. Physiologic lagophthalmos-short upper tarsus in some Asian and some white
persons with incomplete descent of the lid during sleep
7. Supranuclear origin-extrapyramidal syndromes have defective inhibition of lids in
downward gaze
A. Congenital supranuclear lid lag
B. Guillain-Barré syndrome
C. Postencephalitic parkinsonism, Parkinson syndrome (shaking palsy)
D. Progressive supranuclear palsy
Kirkali P, Kansu T. Lid lag in hyperkalemic periodic paralysis. Ann Ophthal 1991;
23:422-423.
Tan E, et al. Lid lag and the Guillain-Barré syndrome. J Clin Neuro-Ophthal 1990;
10:121-123.
BLEPHAROSPASM (SPASMODIC EYELID CLOSURE)
Most common and important: Psychogenic onset commonly in children and young adults
1. Addison disease (adrenal cortical insufficiency)

2. Associated with syphilis, tetanus, and tetany
3. Basal ganglion dysfunction-onset usually after middle age; including Parkinson disease
(shaking palsy)
4. Cerebral palsy
5. Cogan syndrome (nonsyphilitic interstitial keratitis) with vestibuloauditory symptoms
acetophenazine dimercaprol mesoridazine
amitriptyline dimethindene methamphetamine
amodiaquine diphenhydramine methdilazine
amoxapine(?) diphenylpyraline methotrimeprazine
amphetamine doxepin nortriptyline
antazoline doxylamine pentylenetetrazol
brompheniramine dronabinol perazine
butaperazine droperidol pericyazine
carbinoxamine emetine perphenazine
carphenazine ethopropazine pheniramine
chloroquine fluphenazine phenmetrazine
chlorpheniramine haloperidol phenylephrine
chlorpromazine hashish piperacetazine
clemastine hydroxychloroquine prochlorperazine
clomipramine imipramine promazine
desipramine levodopa promethazine
dexbrompheniramine levothyroxine propiomazine
dexchlorpheniramine liothyronine protriptyline
dextroamphetamine liotrix pyrilamine
dextrothyroxine lorazepam selegiline
diethazine marihuana tetrahydrocannabinol
thiethylperazine thyroid trimipramine
thiopropazate trifluoperazine tripelennamine
thioproperazine trifluperidol triprolidine
thioridazine triflupromazine vidarabine
thyroglobulin trimeprazine vinblastine
7. Electrical injury
8. Encephalitis
9. Epidemic keratoconjunctivitis
10. Hallervorden-Spatz
11. Hereditary reflex blepharospasm
12. Idiopathic (essential)
13. Leprosy (Hansen disease)
14. Meige syndrome
15. Obsessive-compulsive disorder
*16. Pain or light sensitivity following injury or inflammation or foreign bodies of lids,
conjunctiva, cornea, or iris
17. Photosensitivity and sunburn
18. Poison ivy dermatitis
19. Postencephalitic blepharospasm
20. Psychogenic obsessive-compulsive disorder-onset commonly in children and young
adults
21. Psychologic reflex blepharospasm-seen in premature infants with tactile stimulation
of lids
22. Sparganosis
23. Systemic scleroderma (progressive systemic sclerosis)
24. Thomsen syndrome (congenital myotonia syndrome)
25. Tourette syndrome (coprolalia, generalized tic)
Defazio G, et al. Genetic contribution to idiopathic adult-onset blepharospasm and

cranial-cervical dystonia. Eur Neurol 1993; 33:345-350.
Larumbe R, et al. Reflex blepharospasm associated with bilateral basal ganglia lesion.
Movement Disorders 1993; 8:198-200.
Patel BC, Anderson RL. Blepharospasm. Ophthalmic Practice 1993; 11:293-302.
FACIAL PALSY
Facial palsy is defined as paralysis of facial muscles supplied by the seventh
nerve; orbicularis oculi paralysis may result in epiphora and ectropion.
1. Congenital
2. Birth injury with nerve crushed at exit of stylomastoid foramen
3. Myogenic paralysis
A. Myotonic atrophia
B. Facioscapulohumeral type of muscular dystrophy
C. Myasthenia gravis (Erb-Goldflam syndrome)
D. Hypokalemia, periodic
E. Curare poisoning
F. Botulism
G. Congenital facial diplegia (Möbius syndrome)
H. Infants, from maternal ingestion of thalidomide
I. Kugelberg-Welander syndrome
4. Neurologic paralysis
A. Supranuclear paralysis-upper face, including orbicularis relatively unaffected
with affected lower face
(1) Voluntary movement-pyramidal fibers involved, such as in Weber
syndrome, with contralateral hemiplegia of face and limbs and ipsilateral
oculomotor paralysis
(2) Weakness or abolition of the emotional movements of the face with
retention of full voluntary activity, such as with lesion of anterior part of
frontal lobe or near optic thalamus
B. Peripheral paralysis-involvement of upper and lower face
(1) Pontine lesion-associated structures involved include sixth nerve,
conjugate ocular deviation to the same side, ipsilateral paralysis of jaw
muscles, and pyramidal tract in paralysis of limb of opposite side
a. Acute nuclear lesions, such as with anterior poliomyelitis,
Landry paralysis, or degenerative conditions
b. Foville syndrome-ipsilateral sixth nerve with loss of conjugate
deviation to same side and hemiplegia of the opposite limbs
c. Millard-Gubler syndrome-ipsilateral sixth nerve paralysis and
hemiplegia of the opposite limbs
d. Parotid gland surgery
e. Progressive muscular atrophy
f. Syringobulbia
g. Tumors
h. Vascular lesions
(2) Posterior fossa-associated with nerve deafness, loss of taste on anterior
two thirds of tongue, and occasionally diminution of tears
a. Acoustic neuroma
b. CHARGE (coloboma, heart disease, atresia choanae, retarded
growth and retarded development or central nervous system
anomalies, genital hypoplasia, and ear anomalies or deafness)
syndrome association
c. Facial neuritis due to polyneuritis cranialis, beriberi,
encephalitis, diabetes, or intrathecal anesthesia
d. Fracture of the skull
e. Meningitis, including syphilitic and tuberculous
f. Preauricular cyst associated with congenital cholesteatoma
g. Tumors of facial nerve
(3) Petrous temporal bone-associated with decreased lacrimation and
salivary secretion, loss of taste on anterior two thirds of tongue, and
intensified sensation of loud noises
*a. Arteriosclerosis
*b. Bell palsy-inflammation of facial nerve of unknown cause
c. Cephalic tetanus
d. Diabetes mellitus (Willis disease)
e. Fractures
f. Herpes zoster, spread from geniculate ganglion
g. Hypertension
* '= most important
h. Nerve leprosy (Hansen disease)
*i. Otitis media
j. Secondary syphilis
(4) Facial lesions at or beyond the stylomastoid foramen
a. Fracture of the ramus of the mandible
b. Melkersson-Rosenthal syndrome (Melkersson idiopathic
fibroedema)
c. Neoplasia or inflammatory swelling of parotid, such as in
uveoparotid fever (Heerfordt disease) and Mikulicz disease
d. Supporting lymph nodes behind the angle of the jaw
Eggenberger ER. Facial palsy in Lyme disease. N Engl J Med 1993; 328:1571.
Lacombe D. Facial palsy and cranial nerve abnormalities in CHARGE association. Am J

Med Genet 1993; 15: 351-353.
INFREQUENT BLINKING
*1. Contact lens use

2. Encephalitis, acute
3. Encephalitis or mild postencephalitic states
*4. Ethanol intake
5. Infants in first few months of life
6. Parkinson syndrome, including mycostatic paresis of parkinsonism
7. Psychotic states
8. Progressive supranuclear palsy
9. Thyrotoxicosis including exophthalmic ophthalmoplegia (Stellwag sign)
Wilkins, 1985.
FREQUENT BLINKING
*1. Reflex-strong lights, sudden approach of objects toward eyes, loud noises, and
touching the cornea; reflex blinking common in albinos and light intolerance
2. Spontaneous-mental state and environment
A. Children with habit spasm and facial tic
B. Blepharospasm
*C. Older persons with inadequate lacrimation and local irritation of the eyes
3. Disorders of central nervous system disease, such as parkinsonism or various forms of
pseudobulbar palsy
acetylcholine chloroquine lidocaine primidone
allobarbital clofibrate mephobarbital probarbital
ambenonium cyclobarbital mepivacaine procaine
amobarbital cyclopentobarbital methacholine propoxycaine
amodiaquine demecarium metharbital pyridostigmine
aprobarbital dibucaine methitural secobarbital
barbital echothiophate methohexital talbutal
bupivacaine edrophonium methylphenidate tetracaine
butabarbital etidocaine neostigmine thiamylal
butalbital heptabarbital pentobarbital thiopental
butallylonal hexethal phenobarbital vinbarbital
butethal hexobarbital physostigmine
carbachol hydroxychloroquine pilocarpine
carbamazepine isoflurophate piperocaine
chloroprocaine levodopa prilocaine
Moses RA. Adler's physiology of the eye, 8th ed. St. Louis: CV Mosby, 1986.
Wilkins, 1985.
LID EDEMA (PUFFINESS OR BAGGINESS OF LIDS)
*1. Noninflammatory or minimally inflammatory swelling

A. Acosta syndrome (Mountain climbers syndrome)
B. Allergic gastroenteropathy with protein loss
C. Arteriovenous fistula
D. Cardiac and renal disease
(1) Nephrosis and acute glomerulonephritis-early morning edema
(2) Starvation and cachexia
E. Dermatochalasis
F. Elephantiasis
(1) Chronic eczema or infection (erysipelas)
(2) Hemolymphangioma
(3) Leprosy (Hansen disease)
(4) Lues (syphilis)
(5) Melkersson-Rosenthal syndrome (Melkersson idiopathic fibroedema)
(6) Nonne-Milroy-Meige disease (idiopathic hereditary lymphedema)
(7) von Recklinghausen disease (neurofibromatosis)
(8) Traumatic disruption of the lymph drainage system
(9) Tuberculosis
G. Endocrine exophthalmos (hyperthyroidism)
H. Foix syndrome (cavernous sinus syndrome)
I. Granulomatous ileocolitis
J. Hutchinson syndrome (adrenal cortex neuroblastoma with orbital metastasis)
K. Infectious generalized diseases
(1) Diphtheria
(2) Infectious mononucleosis
(3) Malaria
(4) Meningococcal meningitis
(5) Pertussis (whooping cough)
(6) Rheumatic fever
(7) Scarlet fever
(8) Trypanosomiasis
(9) Tuberculosis
(10) Yellow fever
L. Melkersson-Rosenthal syndrome
M. Nasal nerve syndrome (Charlin syndrome)
N. Parasitic infestations
(1) Anthrax
(2) Ascariasis
(3) Chlamydia
(4) Dermatophytosis
(5) Myiasis
(6) Onchocerciasis syndrome (river blindness)
(7) Tapeworms
(8) Toxocariasis
(9) Trichinosis
O. Protrusion of fat through orbital fascia
P. Retinoblastoma
Q. Stasis, including premenstrual edema
R. Superior vena cava syndrome
S. Systemic scleroderma (progressive systemic scleroderma)
*T. Tumors and pseudotumors
(1) Benign and malignant ectodermal and mesodermal tumors
(2) Brill-Symmers disease (lymphosarcoma)
(3) Hemangiomas
(4) Hodgkin disease
(5) Leukemic deposit
(6) Liposarcoma
(7) Meningiomas of sphenoid ridge with impediment of venous circulation
of ophthalmic veins or cavernous sinus
(8) Neurofibromatosis
(9) Pseudotumors
a. Amyloid degeneration
b. Eosinophilic or basophilic granulomas
U. Trauma
(1) Basilar skull fractures
(2) Injury
*(3) Surgery
V. Angioneurotic edema caused by drugs, including the following:
acenocoumarol antimony sodium tartrate bupivacaine
acetaminophen antimony sodium busulfan
acetanilid thioglycolate butabarbital
acetophenazine antipyrine butalbital
acetyldigitoxin aprobarbital butallylonal
acyclovir aspirin butaperazine
adrenal cortex injection atropine butethal
albuterol auranofin cactinomycin
alcohol aurothioglucose capreomycin
aldosterone aurothioglycanide captopril
allobarbital azatadine carbamazepine
alprazolam bacitracin carbenicillin
aluminum nicotinate barbital carbimazole
aminosalicylate(?) belladonna carbinoxamine
aminosalicylic acid(?) bendroflumethiazide carisoprodol
amiodarone benzalkonium carphenazine
amitriptyline benzathine penicillin G cefaclor
amobarbital benzphetamine cefadroxil
amoxapine benzthiazide cefamandole
amoxicillin betamethasone cefazolin
ampicillin betaxolol cefonicid
anisindione bleomycin cefoperazone
antazoline botulinum A toxin ceforanide
antimony lithium thiomalate brimonidine tartrate cefotaxime
antimony potassium tartrate brompheniramine cefotetan
cefoxitin cytarabine doxorubicin
cefsulodin dacarbazine doxycycline
ceftazidime dactinomycin doxylamine
ceftizoxime danazol droperidol
ceftriaxone dantrolene echothiophate
cefuroxime dapiprazole hydrochloride emetine
cephalexin dapsone enalapril
cephaloglycin daunorubicin ergonovine
cephaloridine deferoxamine ergotamine
cephalothin demecarium erythromycin
cephapirin demeclocycline ether
cephradine desipramine ethionamide
chloral hydrate deslanoside ethopropazine
chlorambucil desoxycorticosterone ethosuximide
chloramphenicol dexamethasone ethotoin
chlordiazepoxide dexbrompheniramine ethyl biscoumacetate
chloroform dexchlorpheniramine etidocaine
chloroprocaine dextran etretinate
chlorothiazide dextrothyroxine F3T
chlorpheniramine DHT fenfluramine
chlorphentermine diacetylmorphine flecainide
chlorpropamide diatrizoate meglumine and floxuridine
chlorprothixene sodium fludrocortisone
chlortetracycline diazepam fluorescein
chlorthalidone dichlorphenamide fluorometholone
chrysarobin dicloxacillin fluorouracil
ciprofloxacin dicumarol fluphenazine
cisplatin diethazine fluprednisone
clindamycin diethylcarbamazine flurazepam
clofibrate diethylpropion gitalin
clomipramine digitalis glyburide
clonazepam digitoxin gold Au 198
clonidine digoxin gold sodium thiomalate
clorazepate diltiazem gold sodium thiosulfate
cloxacillin dimethindene griseofulvin
cobalt dimethyl sulfoxide guanethidine
codeine diphenadione halazepam
colloidal silver combination diphenhydramine haloperidol
products diphenylpyraline heparin
cortisone diphtheria and tetanus heptabarbital
cyclobarbital toxoids (adsorbed) hetacillin
cyclopentobarbital diphtheria and tetanus hexethal
cyclophosphamide toxoids and pertussis hexobarbital
cyclosporine vaccine (adsorbed) homatropine
cyclothiazide disulfiram hydrabamine penicillin
cyproheptadine doxepin V
hydralazine mercuric oxide paramethadione
hydrocortisone mesoridazine paramethasone
ibuprofen methacycline penicillin
IDU (idoxuridine) metharbital pentazocine
imipramine methdilazine pentobarbital
indomethacin methicillin perazine
insulin methitural pericyazine
iodide and iodine solution methohexital perphenazine
and compounds methotrimeprazine phenacetin
iodipamide meglumine methsuximide phenobarbital
iothalamate meglumine methyl scopolamine phenoxymethyl
iron dextran methyldopa penicillin
isoniazid methylene blue phensuximide
isopropyl unoprostone methylergonovine phenylbutazone
isosorbide methylprednisolone phenylmercuric acetate
isotretinoin methysergide phenylmercuric nitrate
ketoprofen metoclopramide pimozide
lanatoside C metrizamide piperacetazine
latanoprost metronidazole piperazine
leuprolide acetate mexiletine piroxicam
levodopa mianserin poliovirus vaccine
levothyroxine midazolam polymyxin B prazosin
lidocaine minocycline potassium penicillin G
lincomycin mitomycin potassium penicillin V
liothyronine moxalactam potassium phenethicillin
liotrix mumps virus vaccine (live) potassium
lithium carbonate nafcillin phenoxymethyl
lorazepam nalidixic acid prazepam
loxapine naltrexone prednisolone
mannitol naproxen prednisone
maprotiline niacin primidone
measles and rubella virus niacinamide probarbital procaine
vaccine (live) nicotinic acid penicillin G
measles virus vaccine (live) nicotinyl alcohol procaine
measles, mumps, and rubella nifedipine prochlorperazine
virus vaccine (live) nitrazepam promazine
mecamylamine nitrofurantoin promethazine
medroxyprogesterone nitromersol of estrogens propiomazine
medrysone and progestogens propofol
mefenamic acid olapatadine hydrochloride propoxycaine
melphalan oral contraceptives protriptyline
mephenytoin ouabain quinacrine
mephobarbital oxacillin quinidine
mepivacaine oxprenolol quinine
meprednisone oxyphenbutazone rabies immune globulin
meprobamate oxytetracycline rabies vaccine
radioactive iodides talbutal tolbutamide
ranitidine temazepam tretinoin
rifampin tetanus immune globulin triamcinolone
rubella and mumps virus tetanus toxoid triazolam
vaccine (live) tetracycline trichloroethylene
rubella virus vaccine (live) tetraethylammonium trifluoperazine
scopolamine thiabendazole trifluperidol
secobarbital thiamylal triflupromazine
silver nitrate thiethylperazine trifluridine
silver protein thimerosal trimeprazine
smallpox vaccine thiopental trimethadione
sodium antimony gluconate thiopropazate trimethaphan
sodium iothalamate acid thioproperazine trimethidium
isoflurophate thioridazine trimipramine
sodium salicylate thiotepa vancomycin
streptomycin thiothixene verapamil
succinylcholine thyroglobulin vidarabine
sulindac tocainide vinbarbital
suramin tolazamide vinblastine
2. Inflammatory edema
A. Acute hemorrhagic conjunctivitis
B. Allergic eczema (contact dermatitis)
(l) Anesthetics
*(2) Atropine (topical)
(3) Eczematous keratoconjunctivitis
(4) Iodine
(5) Mercury
(6) Neurodermatitis
(7) Penicillin
(8) Photodermatitis
(9) Quincke edema
(10) Tuberculosis (scrofula)
C. Anthrax
D. Bee sting of the cornea
E. Dacryoadenitis
(1) Acute dacryoadenitis
(2) Chronic dacryoadenitis
F. Epidemic keratoconjunctivitis
G. Erysipelas
H. Herpes simplex
I. Hollenhorst syndrome (chorioretinal infarction syndrome)
J. Hordeolum, chalazion
K. Lymphogranuloma venereum
L. Mycoses
M. Ophthalmic zoster
N. Other causes of lid edema
(1) Conjunctival inflammations
a. Diphtheria
b. Newcastle disease (fowlpox)
c. Ophthalmia neonatorum
d. Parinaud syndrome (conjunctiva-adenitis syndrome)
(2) Keratitis
(3) Orbital inflammation
(4) Periostitis
(5) Scleritis (see p. 237-239)
a. Posterior scleritis
b. Scleromalacia perforans
(6) Thermal, chemical, mechanical, or radiation injury
a. Hypothermal injury
b. Polychlorinated biphenyl (PCB)
O. Scalded skin syndrome (Ritter disease)
P. Serum sickness-systemic reaction to foreign serum, serum products, vaccines,
penicillin, and sulfa drugs
Q. Silverman syndrome (battered-baby syndrome)
R. Spider bites
S. Urticaria due to drugs, including the following:
acenocoumarin antimony sodium butallylonal
acetaminophen thioglycollate cactinomycin
acetanilid antipyrine capreomycin
acetazolamide aprobarbital captopril
acyclovir aspirin carbamazepine
albuterol auranofin carbenicillin
allobarbital aurothioglucose carbimazole
allopurinol aurothioglycanide carbinoxamine
alprazolam azatadine carisoprodol
aluminum nicotinate Bacille Calmette-Guérin cefaclor
amiodarone (BCG) vaccine cefadroxil
amitriptyline bacitracin cefamandole
amobarbital barbital cefazolin
amoxapine bendroflumethiazide cefonicid
amoxicillin benzathine penicillin G cefoperazone
ampicillin benzphetamine ceforanide
anisindione benzthiazide cefotaxime
antazoline betamethasone cefotetan
antimony lithium betaxolol cefoxitin
thiomalate bleomycin cefsulodin
antimony potassium brompheniramine ceftazidime
tartrate bupivacaine ceftizoxime
antimony sodium busulfan ceftriaxone
tartrate butabarbital cefuroxime
butalbital cephalexin
cephaloglycin dexchlorpheniramine flecainide
cephaloridine dextran fluorescein
cephalothin diacetylmorphine fluorouracil
cephapirin diatrizoate meglumine flurazepam
cephradine and sodium flurbiprofen
chlorambucil diazepam framycetin
chloramphenicol dichlorphenamide furosemide
chlordiazepoxide dicloxacillin gentamicin
chloroprocaine dicumarol glutethimide
chlorothiazide diethylcarbamazine gold Au 198
chlorpheniramine diethylpropion gold sodium thiomalate
chlorphentermine digitalis gold sodium thiosulfate
chlorporthixene diltiazem griseofulvin
chlortetracycline dimethindene halazepam
chlorthalidone dimethyl imidazole heparin
cimetidine carboxamide (DIC) heptabarbital
cisplatin dimethyl sulfoxide hetacillin
clemastine (DMSO) hexethal
clindamycin diphenadione hexobarbital
clofibrate diphenhydramine hydrabamine penicillin
clomiphene diphenylpyraline V
clomipramine diphtheria and tetanus hydralazine
clonazepam toxoids (adsorbed) hydrochlorothiazide
clonidine diphtheria and tetanus hydrocortisone
clorazepate toxoids and pertussis hydroflumethiazide
cloxacillin (DPT) vaccine hydromorphone
cobalt (adsorbed) ibuprofen
codeine diphtheria toxoids imipramine
cyclobarbital (adsorbed) indapamide
cyclopentobartibal disulfiram indomethacin
cyclophosphamide doxepin influenza virus vaccine
cyclosporine doxorubicin insulin
cyclothiazide doxycycline interferon
cyproheptadine doxylamine iodide and iodine
cytarabine DPT vaccine solutions and
dacarbazine emetine compounds
dactinomycin enalapril iodipamide meglumine
danazol erythromycin iophendylate
dantrolene ethionamide iothalamate meglumine
dapsone ethotoin and sodium
daunorubicin ethoxzolamide iothalamic acid
deferoxamine ethyl biscoumacetate iron dextran
demeclocycline etidocaine isoniazid
desipramine etretinate isosorbide
dexamethasone fenfluramine isotretinoin
dexbrompheniramine fenoprofen ketoprofen
labetalol mianserin poliovirus vaccine
levallorphan midazolam polythiazide
levobunolol minocycline potassium penicillin G
lidocaine mitomycin potassium penicillin V
lincomycin morphine potassium phenethicillin
lorazepam moxalactam practolol
loxapine mumps virus vaccine prazepam
mannitol (live) prazosin
maprotiline nafcillin prilocaine
measles and rubella nalidixic acid primidone
virus vaccine (live) nalorphine probarbital
measles virus vaccine naloxone procaine
(live) naltrexone procaine penicillin G
measles, mumps, and naproxen procarbazine
rubella virus vaccine neomycin propoxycaine
(live) neostigmine propranolol
melphalan niacin propylthiouracil
meperidine niacinamide protriptyline
mephenytoin nicotinyl alcohol pyrilamine
mephobarbital nifedipine quinacrine
mepivacaine nitrazepam quinethazone
meprobamate nitrofurantoin quinidine
mercuric oxide nitromersol quinine
methacycline nortriptyline rabies immune globulin
methadone opium rabies vaccine
methaqualone oral contraceptives radioactive iodides
metharbital oxacillin ranitidine
methazolamide oxazepam rifampin
methicillin oxymorphone rubella and mumps virus
methimazole oxyphenbutazone vaccine (live)
methitural oxytetracycline rubella virus vaccine
methocarbamol penicillamine (live)
methohexital pentazocine secobarbital
methotrexate pentobarbital smallpox vaccine
methyclothiazide phenacetin sodium
methyldopa phendimetrazine antimonylgluconate
methylphenidate phenindione sodium salicylate
methylprednisolone pheniramine stibocaptate
methylthiouracil phenobarbital stibogluconate
methyprylon phenprocoumon stibophen
metoclopramide phentermine streptomycin
metocurine iodide phenylbutazone succinylcholine
metolazone phenylmercuric acetate sulfacetamide
metoprolol phenylmercuric nitrate sulfachlorpyridazine
metrizamide piperazine sulfacytine
metronidazole piroxicam sulfadiazine
sulfadimethoxine sulindac timolol
sulfamerazine suramin triamcinolone
sulfameter talbutal triazolam
sulfamethazine temazepam trichlormethiazide
sulfamethizole tetanus immune globulin trimethaphan
sulfamethoxazole tetanus toxoid trimipramine
sulfamethoxypyridazine tetracycline tripelennamine
sulfanilamide thiabendazole triprolidine
sulfaphenazole thiamylal tubocurarine
sulfapyridine thimerosal vancomycin
sulfasalazine thiopental verapamil
sulfathiazole thiotepa vinbarbital
sulfisoxazole thiothixene warfarin
T. Wegener syndrome (Wegener granulomatosis)

U. Vaccination
(1) Ocular vaccina
(2) Postvaccinial ocular syndrome
(3) Variola
Cockerham KP, et al. Melkersson-Rosenthal syndrome. Arch Ophthalmol 2000; 118:227-

230.
Newell F. Ophthalmology, principles and concepts, 7th ed. St. Louis: CV Mosby, 1991.
Yeatts RP, White WL. Granulomatous blepharitis as a sign of Melkersson-Rosenthal

BLEEDING OF THE EYELID

acetazolamide aurothioglycanide chlorpropamide
acetohexamide BCG vaccine chlorthalidone
allopurinol bendroflumethiazide cimetidine
alprazolam benzthiazide clofibrate
amantadine betamethasone clonazepam
amitriptyline betaxolol clorazepate
aspirin carbamazepine cyclothiazide
auranofin chlordiazepoxide cytarabine
aurothioglucose chlorothiazide danazol
dapsone measles, mumps, and rubella virus vaccine
desipramine rubella virus vaccine (live)
dexamethasone (live) smallpox vaccine
diazepam methaqualone sodium salicylate
dichlorphenamide methazolamide sulfacetamide
diltiazem methyclothiazide sulfachlorpyridazine
emetine methylprednisolone sulfacytine
ethoxzolamide methyprylon sulfadiazine
flurazepam metolazone sulfadimethoxine
furosemide metoprolol sulfamerazine
glutethimide midazolam sulfameter
glyburide mumps virus vaccine (live) sulfamethazine
gold Au 198 naproxen sulfamethizole
gold sodium thiomalate nifedipine sulfamethoxazole
gold sodium thiosulfate nitrazepam sulfamethoxypyridazine
halazepam nortriptyline sulfanilamide
hydrochlorothiazide oxazepam sulfaphenazole
hydrocortisone oxprenolol sulfapyridine
hydroflumethiazide phenytoin sulfasalazine
ibuprofen piperazine sulfathiazole
imipramine piroxicam sulfisoxazole
indapamide polythiazide temazepam
indomethacin prazepam tetanus immune globulin
influenza virus vaccine procarbazine tetanus toxoid
interferon propranolol timolol
ketoprofen protriptyline tolazamide
levobunolol quinethazone tolbutamide
lorazepam quinine triamcinolone
measles and rubella virus rifampin triazolam
vaccine (live) rubella and mumps virus trichlormethiazide
measles virus vaccine (live) vaccine (live) verapamil
2. Hutchinson syndrome (adrenal cortex neuroblastoma with orbital metastasis)

*3. Trauma
ECTROPION (LID MARGIN TURNED OUTWARD FROM THE EYEBALL)
1. Congenital ectropion
A. With distichiasis
B. With tight septum; microblepharon
C. With partial coloboma
D. With mandibulofacial dysostosis (Franceschetti syndrome)
E. With megaloblepharon (euryblepharon)
F. With microphthalmos or buphthalmos
G. Cerebrooculofacioskeletal syndrome
H. Down syndrome (mongolism)
I. Hartnup syndrome (niacin deficiency)
J. Lowe syndrome (oculocerebrorenal syndrome)
K. Miller syndrome
L. Milroy disease (oromandibular dystonia)
M. Robinow syndrome
N. Sjögren-Larrson syndrome
2. Acquired ectropion
A. Spastic ectropion
*(1) Acute spastic ectropion
(2) Blepharophimosis syndrome
*(3) Chronic spastic ectropion becoming cicatricial ectropion
(4) Hypothermal injury
(5) Myasthenia gravis-afternoon onset (Erb-Goldflam syndrome)
(6) Siemen syndrome (hereditary ectodermal dysplasia syndrome)
B. Atonic ectropion
(1) Anophthalmic socket
(1) Bell palsy (Idiopathic facial paralysis)
(2) Guillain-Barré syndrome (acute infectious neuritis)
(3) Paralytic ectropion-lagophthalmos, such as in seventh nerve palsy
*(4) Senile ectropion-tissue relaxation
C. Cicatricial ectropion
(1) Amendola syndrome
(2) Blastomycosis
(3) Collodion baby syndrome (congenital ichthyosis)
(4) Chronic dermatitis
(5) Cutaneous T-cell
(6) Etretinate therapy
(7) Excessive skin excision
(8) Facial bums and scarring
(9) Hydroa vacciniforme
(10) Kabuki makeup syndrome
(12) Orbital fracture repair
(13) Palmoplantar keratodermia
(14) Postblepharoplasty ectropion
(15) Psoriasis (psoriasis vulgaris)
(16) Radiation
(17) Sézary syndrome (malignant cutaneous reticulosis syndrome)
(18) Systemic fluorouracil
(19) Thermal bums
(20) Trauma
(21) Transformation from chronic spastic ectropion
(22) Zinsser-Engman-Cole syndrome (dyskeratosis congenita with
pigmentation)
D. Allergic ectropion-anaphylactic, contact, and microbial (usually temporary)
(1) Danbolt-Closs syndrome (acrodermatitis enteropathica)
(2) Elschnig syndrome
E. Mechanical
(1) Kaposi disease (multiple idiopathic hemorrhagic sarcoma)
(2) Leiomyoma
(3) Lumps (chalazion, cysts, neurofibroma)
Hurwitz BS. Cicatricial ectropion: a complication of systemic fluorouracil. Arch Ophthal

1993; 11l: 1608-1609.
Stratakis CA, et al. A variant of the cerebro-ocu1o-facio-skeletal syndrome with

congenital ectropion and a case of lamellar ichthyosis in the same family. Clin Genet
1994; 45:162-163.
ENTROPION (INVERSION OF LID MARGIN)
1. Congenital, including congenital epiblepharon-inferior oblique insufficiency;

ectrodactyly, ectodermal dysplasia, cleft lip-palate syndrome, including with and without
lower eyelid retractor insertion
A. Inferior oblique insufficiency syndrome
B. Dental-ocular-cutaneous syndrome
C. Siemen syndrome (anhidrotic ectodermal dysplasia)
2. Acquired
*A. Spastic entropion-acute, affecting lower lid, precipitated by acute
inflammation or prolonged patching
B. Mechanical entropion
(1) Anophthalmos
(2) Enophthalmos
(3) Microphthalmos
(4) Lymphedema
*C. Senile entropion-relative enophthalmos secondary to fat atrophy
D. Cicatricial entropion-physical and chemical bums of conjunctiva and
cicatrizing diseases, including trachoma and leprosy
(1) Chronic cicatricial conjunctivitis
(3) Radiation
(4) Thermal bums
(5) Trachoma
(6) Following cryosurgery of the eyelid
(7) Amendola syndrome
(8) Variola
Bartley GB, et al. Congenital entropion with intact lower eyelid retractor insertion. Am J
Ophthalmol 1991; 112: 437-441.
Westfall CT, et al. Operative complications of the transconjunctival inferior fornix

approach. Ophthalmology 1991; 98: 1525-1528.
EPICANTHUS (FOLD OF SKIN OVER INNER CANTHUS OF EYE)
1. Types
A. Epicanthus inversus-fold arises in the lower lid and extends upward to a point
slightly above the inner canthus; it is accompanied by long medial canthal
tendons, blepharophimosis, and ptosis-autosomal dominant
*B. Epicanthus palpebralis (common type)-epicanthal fold arises from the upper
lid above the tarsal region and extends to the lower margin of the orbit
C. Epicanthus supraciliaris (unusual type)-epicanthal fold arises near brow and
runs toward tear sac
*D. Epicanthus tarsalis (Mongolian eye)-epicanthal fold arises from the tarsal
(lid) fold and loses itself in the skin close to the inner canthus-autosomal
dominant
2. Associated conditions
A. Aminopterin-induced syndrome
B. Basal cell nevus syndrome (Gorlin syndrome)
C. Bassen-Kornzweig syndrome (familial hypolipoproteinemia)
D. Bilateral renal agenesis
E. Blepharophimosis, ptosis, epicanthus inversus syndrome
F. Bonnevie-Ullrich syndrome (pterygolymphangiectasia)
G. Carpenter syndrome (acrocephalopolysyndactyly II)
H. Cat-eye syndrome (partial G-trisomy syndrome)
I. Cerebrofacioarticular syndrome of van Maldergen
J. Cerebrohepatorenal syndrome (Smith-Lemli-Opitz syndrome)
K. Chondrodystrophia (Conradi syndrome)
L. Chromosome long-arm deletion syndrome
M. Chromosome deletion (deletion 18)
N. Chromosome partial short-arm deletion syndrome (Wolf syndrome)
O. Chromosome short-arm deletion syndrome
P. Chromosome 13q partial deletion syndrome
Q. Congenital facial paralysis (Möbius syndrome)
R. Craniocarpotarsal syndrome (whistling face syndrome)
S. Craniosynostosis-radial aplasia (Baller-Gerold syndrome)
T. Cri-du-chat syndrome (Cry of the cat syndrome)
U. Dubowitz syndrome
V. Down syndrome (trisomy 21, mongolism)
W. Drummond syndrome (idiopathic hypercalcemia, blue diaper syndrome)
X. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
Y. 18q syndrome
Z. Familial blepharophimosis
AA. Fetal alcohol syndrome
BB. Freeman-Sheldon syndrome (whistling face syndrome)
CC. 4Q syndrome
DD. Gansslen syndrome (hematologic-metabolic bone disorder)
EE. Greig syndrome (ocular hypertelorism syndrome)
FF. Hurler syndrome (dysostosis multiplex)
GG. Infantile hypercalcemia
HH. Jacobs syndrome (triple X syndrome)
II. Klinefelter XXY syndrome (gynecomastia-aspermatogenesis syndrome)
JJ. Kohn-Romano syndrome (ptosis, blepharophimosis, epicanthus inversus, and
telecanthus)
KK. Komoto syndrome (congenital eyelid tetrad)
LL. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-
adiposogenital)
MM. Leopard syndrome (multiple lentigines syndrome)
NN. Leroy syndrome (mucopolysaccharide excretion)
OO. Little syndrome (nail patella syndrome)
PP. Michel syndrome
QQ. Mohr-Claussen syndrome (similar to orodigitofacial syndrome)
RR. Noonan syndrome (Turner syndrome in males)
SS. Oculocerebrorenal syndrome (Lowe syndrome)
TT. Oculodentodigital dysplasia (microphthalmos syndrome)
UU. Potter syndrome (renofacial syndrome)
VV. Ring chromosome syndrome
WW. Ring chromosome syndrome
XX. Ring chromosome (microcephaly, hypertelorism, epicanthus)
YY. Ring chromosome in the D group (13-15)
ZZ. Robinow-Silverman-Smith syndrome
AAA. Rubinstein-Taybi syndrome (broad thumbs syndrome)
BBB. Schonenberg syndrome (dwarf-cardiopathy syndrome)
CCC. Smith syndrome (facioskeletogenital dysplasia)
DDD. TAR (thrombocytopenia absent radius) syndrome
EEE. Thalassemia
FFF. Trisomy syndrome (Edward syndrome)
GGG. Turner syndrome (gonadal dysgenesis)
HHH. Waardenburg syndrome (embryonic fixation syndrome)
III. X-linked mental retardation syndrome
JJJ. XXXXX syndrome
HYPOPIGMENTATION (DEPIGMENTATION OF EYELIDS)

adrenal cortex injection dexamethasone methotrexate
alcohol fludrocortisone methylprednisolone
aldosterone fluorometholone methylthiouracil
beclomethasone fluprenisolone neostigmine
amodiaquine gentamicin(?) paramethasone
arsenic hydrocortisone physostigmine
betamethasone hydroquinone prednisolone
carbimazole hydroxychloroquine prednisone
chloramphenicol isoflurophate propylthiouracil
chloroquine medrysone thiotepa
corticosteroids meprednisone triamcinolone
cortisone mercaptoethylamine
desoxycortiscosterone methimazole
2. Genetic factors
A. Albinism
B. Chediak-Higashi syndrome (anomalous leukocytic inclusions with
constitutional stigmata)
C. Cross-McKusick-Breen syndrome
D. Fanconi syndrome (amino diabetes)
E. Hermansky-Pudlak syndrome (oculocutaneous albinism and hemorrhagic
diathesis)
F. Histidinemia
G. Homocystinuria
H. Incontinentia pigmenti achromians (hypomelanosis of Ito syndrome)
I. Menkes syndrome (kinky hair syndrome)
J. Nevus depigmentosus
K. Phenylketonuria (Folling syndrome)
L. Tuberous sclerosis (Bourneville syndrome)
M. Vitiligo
N. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis syndrome)
O. Waardenburg syndrome (embryonic fixation syndrome)
P. Woolf syndrome (chromosome partial deletion syndrome)
Q. Ziprkowski-Margolis syndrome
3. Following cryosurgery of the eyelid
4. Bums (thermal, ultraviolet, ionizing, radiation)
5. Trauma
6. Kwashiorkor-malnutrition in children
7. Chronic protein deficiency or loss and malabsorption of vitamin B12
8. Endocrine factors
A. Hypopituitarism (Simmond syndrome)
B. Addison disease (adrenal cortical insufficiency)
C. Hyperthyroidism (Graves disease)
9. Inflammation and infection
A. Discoid lupus erythematosus
B. Eczematous dermatitis
C. Leprosy (Hansen disease)
D. Onchocerciasis syndrome (river blindness)
E. Pinta
F. Pityriasis alba
G. Postinflammatory hypomelanoses
H. Post-kala-azar
I. Psoriasis
J. Sarcoidosis syndrome (Schaumann syndrome)
K. Syphilis (acquired lues)
L. Tinea versicolor
M. Vagabond leukoderma
N. Vitiligo
O. Yaws
HYPERPIGMENTATION (DISCOLORATION OF LIDS)
1. Deposits of the eyelids as caused by drugs, including:

2. Hyperpigmentation as caused by drugs, including the following:
acetophenazine bismuth oxychloride carphenazine
acid bismuth sodium bismuth sodium tartrate chlorpromazine
tartrate bismuth sodium chlortetracycline
actinomycin C thioglycollate chrysarobin
Alcian blue bismuth sodium clofazimine
aluminum nicotinate triglycollamate colloidal silver
aminopterin bismuth sub salicylate cyclophosphamide
amiodarone chloroquine cytarabine
amodiaquine bismuth subcarbonate dactinomycin
amphotericin B bleomycin demeclocycline
antipyrine busulfan diethazine
aurothioglucose butaperazine doxycycline
aurothioglycanide calcitriol dromostanolone
enalapril methotrexate propiomazine
epinephrine methotrimeprazine quinacrine
ergocalciferol methylene blue rifampin
ethopropazine mild silver protein rose bengal
ferrocholinate minocycline silver nitrate
ferrous fumarate minoxidil silver protein
ferrous gluconate niacinamide sulfacetamide
ferrous succinate nicotinic acid sulfamethizole
ferrous sulfate nicotinyl alcohol sulfisoxazole
floxuridine nitromersol testolactone
fluorescein oxytetracycline testosterone
fluorouracil penicillamine tetracycline
fluoxymesterone perazine thiethylperazine
fluphenazine pericyazine thimerosal
gold Au 198 perphenazine thimerosal thiopropazate
gold sodium thiomalate phenylmercuric acetate thioguanine
gold sodium thiosulfate phenylmercuric nitrate thioproperazine
hydroxychloroquine piperacetazine thioridazine
iron dextran pipobroman trifluoperazine
iron sorbitex polysaccharide-iron triflupromazine
ketoprofen complex trimeprazine
mercaptopurine practolol uracil mustard
mercuric oxide procarbazine vitamin A
mesoridazine prochlorperazine vitamin D2
methacycline promazine vitamin D3
methdilazine promethazine
3. Periorbital hyperpigmentation-dark circles around the eye

*A. Allergic rhinitis
*B. Familial (autosomal dominant)
C. Medium and dark complexioned white persons
4. Brown hyperpigmentation
A. Genetic factors
(1) Acanthosis nigricans
(2) Albright syndrome (fibrous dysplasia)
(3) Cafe-au-lait and freckle-like macules in neurofibromatosis
(4) Dyskeratosis congenita
(5) Fanconi syndrome (amino diabetes)
(6) Freckles
(7) Lentigines
(8) Melanocytic nevus
(9) Neurocutaneous melanosis
(10) Seborrheic keratosis
(11) Xeroderma pigmentosum
B. Metabolic factors
(1) Gaucher syndrome (cerebroside lipidosis)
(2) Hemochromatosis
(3) Niemann-Pick disease (essential lipoid histiocytosis)
(4) Porphyria (cutanea tarda)
(5) Wilson disease (hepatolenticular degeneration)
C. Endocrine factors
(1) adrenocorticotropic hormone (ACTH) therapy
(2) Addison disease (adrenal cortical insufficiency)
(3) Estrogen therapy
(4) Melanoma
(5) Pituitary tumors
(6) Pregnancy
D. Nutritional factors
(1) Kwashiorkor (hypoproteinemia syndrome)
(2) Pellagra (avitaminosis B2)
(3) Sprue
(4) Vitamin B12 deficiency (Addison pernicious anemia)
E. Chemical and pharmacologic agents
(1) Arsenic
(2) Berlock dermatosis
(3) Bleomycin
(4) Busulfan
(5) Nitrogen mustard, topical
(6) Photochemical agents
F. Physical agents
(1) Ionizing radiation
(2) Thermal radiation
*(3) Trauma
(4) Ultraviolet light
G. Inflammation and infection
(1) Atopic dermatitis
(2) Lichen planus
(3) Lichen simplex chronicus
(4) Lupus erythematosus discoid (Kaposi-Libman-Sacks syndrome)
(5) Psoriasis
(6) Tinea versicolor
H. Neoplasms
(1) Acanthosis nigricans
(3) Mastocytosis
I. Miscellaneous factors
(1) Autosomal recessive ectodermal dysplasia
(2) Catatonic schizophrenia
(3) Chronic hepatic insufficiency
(4) Cronkhite-Canada syndrome
(5) Encephalitis
(6) Erythema dyschromicum perstans
(7) Liver spots
(8) Systemic scleroderma (progressive systemic sclerosis)
(9) Whipple syndrome (intestinal lipodystrophy)
5. Blue, gray or slate hyperpigmentation
A. Genetic factors
(1) Blue melanocytic nevus
(2) Dermal melanocytosis (Mongolian spot)
(3) Franceschetti-Jadassohn syndrome (reticular pigmented dermatosis)
(4) Incontinentia pigmenti (Bloch-Sulzberger syndrome)
(5) Oculodermal melanocytosis
B. Metabolic factors
(1) Amyloidosis, cutaneous macular (Lubarsch-Pick syndrome)
(2) Hemochromatosis
C. Nutritional factors
(1) Chronic nutritional insufficiency
D. Inflammation and infection
(1) Erythema dyschromicum perstans
(2) Pinta
(3) Riehl melanosis
E. Chemical and pharmacologic agents
(1) Chlorpromazine
(2) Gold
(3) Phenothiazine
(4) Sulfonamides
(5) Tetracycline
F. Neoplasms
(1) Slate-gray dermal pigmentation with metastatic melanoma and
melanogenemia
G. Other
(1) Blue dye
(2) Cyanosis
Haddock N, Wilkin JK. Periorbital hyperpigmentation. JAMA 1981; 246:835.
Pau H. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical, 1988.
Schwartz MP. Blue Christmas. JAMA 1987; 257:3229-3230.
TUMORS OF EYELIDS
1. Molluscum contagiosum-small, greasy-appearing elevation that is usually umbilicated

or any other granuloma
2. Neoplasm
A. Basal cell epithelioma-common; may be a red, circumscribed, lobulated
growth involving the lid margin or may have an umbilicated center (rodent ulcer)
B. Squamous cell or Zeis cell epithelioma-hard pearly appearing lesion, usually
without increased vascularity
C. Meibomian-gland carcinoma-resembles a chalazion
D. Metastatic tumors of the lid-respiratory tract, breast, skin (melanoma),
gastrointestinal tract, or kidney
E. Keratoacanthoma-benign, hemispherical, elevated tumor with a central keratin-
filled crater; develops within several months
F. Hemangioma - rubor of vascular tumor, usually having a smooth surface with
tufts of vessels near the surface
G. Benign mixed tumor of the lacrimal (palpebral) gland
H. Trichilemmoma
I. Lymphangioma
J. Juvenile xanthogranuloma
3. Metaplasia or hyperplasia
A. Trichoepithelioma
B. Syringoma
*C. Sebaceous adenoma
*D. Papilloma-smooth, rounded, or pedunculated elevation
*E. Nevus-usually pigmented, raised, and smooth surfaced; however, may be
papillomatous or contain hair
F. Benign calcifying epithelioma
G. Inverted follicular keratosis
H. Blue nevus-blue-black and velvet-like in appearance
I. Freckles
J. Lentigo simplex
K. Solar lentigo
L. Melasma
Extracted Table Tumors of eyelids
4. Cyst
*A. Sebaceous
B. Sudoriferous
C. Traumatic
D. Congenital inclusion
5. Lipoid proteinosis-wax-like, pearly nodules
6. Pseudotumor of lid-encysted contact lens
Boynton JR, Markowitch W. Porocarcinoma of eyelid. Ophthalmology 1997; 104:1626-

1628.
Cook BE, Bartley GB. Epidemiologic characteristics and clinical course of patients with
malignant eyelid tumors in an incidence cohort in Olmsted County, Minnesota.
Kerten RC, et al. Accuracy of clinical diagnosis of cutaneous eyelid lesions.

XANTHELASMA (SMOOTH YELLOW DEPOSITS IN THE EYELID,

ESPECIALLY THE SUPERIOR NASAL AND INFERIOR NASAL AREAS)
1. Xanthelasma with hyperlipemia (primary or secondary)

*A. Type II-familial hyper-B-lipoproteinemia (familial hypercholesterolemia)
*B. Type III-familial hyper-B- and hyper-pre-B-lipoproteinemia (familial
hyperlipemia with hypercholesterolemia)
C. Other types are infrequent, including type I, familial fat-induced
hyperlipoproteinemia (hyperchylomicronemia); type IV, familial hyper-pre-B-
lipoproteinemia (carbohydrate-induced hyperlipemia); type V, familial
hyperchylomicronemia with hyper-pre-B-lipoproteinemia (mixed hyperlipemia), lichen
sclerosis et atrophicus.
2. Xanthelasma without hyperlipemia
A. Generalized
B. Histiocytosis X (eosinophilic granuloma, Hand-Schüller-Christian disease, and
Letterer-Siwe disease)
C. Local (no systemic disease)
D. Reticulohistiocytoma cutis
E. Xanthoma disseminatum
Depot MJ, et al. Bilateral and extensive xanthelasma palpebrarum in a young man.
CHRONIC BLEPHARITIS (INFLAMMATION OF LIDS)
*1. Seborrheic-lid margin covered with small, white or gray scales

A. Associated with seborrheic dermatitis of the scalp
B. Aggravated by chemical fumes, smoke, and smog
C. May be associated with uncorrected refractive errors (especially hyperopia)
D. May be due to Pityrosporon ovale
E. Aspergillus fumigatus may be its cause
F. Associated with systemic diseases
(1) Acne rosacea
(2) Acinetobacter Iwoffi
(3) Acrodermatitis chronica atrophicans
(4) Aspergillosis
(5) Candidiasis
(6) Cretinism (hypothyroidism)
(7) Demodicosis
(8) Dermatophytosis
(9) Diphtheria
(10) Erysipelas
(11) Herpes simplex
(12) Hypocalcemia
(13) Hypoparathyroidism
(14) Listerellosis
(15) Malaria
(16) Moraxella lacunata
(17) Pellagra (avitaminosis B2)
*(18) Seborrheic dermatitis
(19) Sporotrichosis
(20) Staphylococcus
(21) Streptococcus
(23) Scleroderma (systemic scleroderma)
(24) Tuberculosis
(25) Vaccinia
(26) Xeroderma pigmentosum
G. Associated with syndromes
(2) Down syndrome (mongolism)
(3) Goldscheider syndrome (epidermolysis bullosa)
(4) Hand-Schüller-Christian syndrome (lipoid granuloma syndrome)
(5) Lyell syndrome (toxic epidermal necrolysis)
(6) Myotonic dystrophy syndrome (Curschmann-Steinert syndrome)
(7) Parkinson syndrome (paralysis agitans)
(8) Sézary syndrome (mycosis fungoides syndrome)
(9) Siemens syndrome (hereditary ectodermal dysplasia syndrome)
(10) Syndrome of Beal (acute follicular conjunctivitis)
(11) Wernicke syndrome
(12) Thiamine deficiency
(13) Wiskott-Aldrich syndrome
(14) Zinsser-Engman-Cole syndrome (dyskeratosis congenita with
pigmentation)
H. Drugs, including the following:
acyclovir meperidine thimerosal
benzalkonium mercuric oxide trifluridine
F3T idoxuridine nitromersol vidarabine
isosorbide phenylmercuric acetate
mannitol phenylmercuric nitrate
2. Ulcerative-suppurative inflammation of the follicles of the lashes and the associated
glands of Zeis and Moll
A. Staphylococcus aureus or S. albus may be responsible
B. Due to mixed infection of a staphylococcus and P. ovale
C. Associated with vaccinia
D. Due to Blastomyces dermatitidis
E. Herpes simplex-vesicles at lash line, then ulceration
*3. Angular-inflammation of the angles of the lids, usually associated with an angular
conjunctivitis
A. Candida albicans
B. Moraxella lacunata
C. Stannus cerebellar syndrome (riboflavin deficiency)
D. Staphylococcus aureus
4. Exfoliative dermatitis owing to drugs, including the following:
acetohexamide bismuth sodium cefuroxime
acetophenazine triglycollamate cephalexin
acid bismuth sodium bismuth subcarbonate cephaloglycin
tartrate bismuth subsalicylate cephaloridine
adiphenine bupivacaine cephalothin
allobarbital busulfan cephapirin
allopurinol butabarbital cephradine
ambutonium butalbital chlorambucil
aminosalicylate(?) butallylonal chloroprocaine
aminosalicylic acid butaperazine chloroquine
amithiozone butethal chlorpromazine
amobarbital captopril chlorpropamide
amodiaquine carbamazepine chlorprothixene
amoxicillin carbenicillin cimetidine
ampicillin carbimazole ciprofloxacin
anisindione carisoprodol clidinium
anisotropine carphenazine clindamycin
antipyrine cefaclor cloxacillin
aprobarbital cefadroxil codeine
atropine methylnitrate cefamandole cyclobarbital
auranofin cefazolin cyclopentobarbital
aurothioglucose cefonicid cyclophosphamide
aurothioglycanide cefoperazone dapsone
barbital ceforanide dicloxacillin
bismuth carbonate cefotaxime dicyclomine
bismuth oxychloride cefotetan diethazine
bismuth salicylate cefoxitin diltiazem
bismuth sodium tartrate cefsulodin diphemanil
bismuth sodium ceftazidime diphenadione
thioglycollate ceftizoxime diphenylhydantoin
ceftriaxone droperidol
enalapril metharbital prochlorperazine
erythrityl tetranitrate methdilazine promazine
erythromycin methicillin promethazine
ethionamide methimazole propantheline
ethopropazine methitural propiomazine
ethosuximide methixene propoxycaine
ethotoin methohexital propoxyphene
etidocaine methotrimeprazine propranolol
fenoprofen methsuximide propylthiouracil
flecainide methylatropine nitrate quinacrine
fluphenazine methylphenidate quinidine
furosemide methylthiouracil radioactive iodides
glutethimide methyprylon rifampin
glyburide moxalactam secobarbital
glycopyrrolate nafcillin streptomycin
gold Au 198 naltrexone sulfacetamide
gold sodium hyposulfite naproxen(?) sulfachlorpyridazine
gold sodium thiomalate nitroglycerin sulfacytine
griseofulvin oxacillin sulfadiazine
haloperidol oxyphenbutazone sulfadimethoxine
heptabarbital oxyphencyclimine sulfamerazine
hetacillin oxyphenonium sulfameter
hexethal paramethadione sulfamethazine
hexobarbital pentaerythritol sulfamethizole
hexocyclium tetranitrate sulfamethoxazole
hydroxychloroquine pentobarbital sulfamethoxypyridazine
indomethacin perazine sulfanilamide
iodide and iodine pericyazine sulfaphenazole
solutions and perphenazine sulfapyridine
compounds phenindione sulfasalazine
isoniazid phenobarbital sulfathiazole
isopropamide phensuximide sulfisoxazole
isosorbide dinitrate phenylbutazone sulindac
ketoprofen phenytoin talbutal
lidocaine pimozide thiabendazole
lincomycin pipenzolate thiamylal
mannitol hexanitrate piperacetazine thiethylperazine
mechlorethamine piperidolate thiopental
melphalan piroxicam thiopropazate
mepenzolate poldine thioproperazine
mephenytoin practolol thioridazine
mephobarbital prilocaine thiothixene
mepivacaine primidone tolazamide
meprobamate probarbital tolbutamide
mesoridazine procaine trichloroethylene
methantheline procarbazine tridihexethyl
triethylenemelamine trimeprazine vancomycin
trifluoperazine trimethadione vinbarbital
trifluperidol trolnitrate vitamin A
triflupromazine uracil mustard
5. Other types
A. Due to mites (Demodex folliculorum)
B. Due to pubic lice (Phthirus pubis)
6. Erythema due to drugs, including the following:
acebutolol cefamandole cyclosporin
acetaminophen cefazolin cyproheptadine
acetanilid cefonicid cytarabine
acetazolamide cefoperazone dacarbazine
acyclovir ceforanide dactinomycin
adrenal cortex injection cefotaxrrne danazol
albuterol cefotetan dapiprazole
aldosterone cefoxitin hydrochloride
allopurinol cefsulodin daunorubicin
alprazolam ceftazidime deferoxamine
amitriptyline ceftizoxime demeclocycline
amoxapine ceftriaxone desipramine
antazoline cefuroxime desoxycorticosterone
atenolol cephalexin dexamethasone
auranofin cephaloglycin dexbrompheniramine
aurothioglucose cephaloridine dexchlorpheniramine
aurothioglycanide cephalothin dextran
azatadine cephapirin diacetylmorphine
BCG vaccine cephradine diatrizoate
beclomethasone chlorambucil diazepam
benzalkonium chlordiazepoxide diazoxide
benzathine penicillin G chloroethyl-cyclohexyl- DIC
benzphetamine nitrosourea (CCNU) dichlorphenamide
betamethasone chlorpheniramine diethylcarbamazine
betaxolol chlorphentermine diethylpropion
Bis-chloroethyl-nitroso- chlorphentermine diltiazem
urea (BCNU) chlortetracycline dimethindene
bleomycin cimetidine dimethyl sulfoxide
bromide ciprofloxacin diphenhydramine
bromphenrramine cisplatin diphenylpyraline
busulfan clemastine diphtheria and tetanus
cactinomycin clofibrate toxoids (adsorbed)
captopril clomipramine diphtheria and tetanus
carbinoxamine clonazepam toxoids and pertussis
carmustine clorazepate vaccine (adsorbed)
cefaclor cortisone diphtheria toxoid
cefadroxil cyclophosphamide (adsorbed)
disopyramide levobunolol neostigmine
disulfiram lomustine nifedipine
DMSO lorazepam nitrazepam
doxepin maprotiline nitromersol
doxorubicin measles and rubella nortriptyline
doxycycline virus vaccine (live) oxazepam
doxylamine measles virus vaccine oxprenolol
DPT vaccine (live) oxytetracycline
enalapril measles, mumps, and paramethasone
ergonovine rubella virus vaccine pentazocine
ergotamine (live) phenacetin
ethionamide mechlorethamine phendimetrazine
ethoxzolamide medrysone pheniramine
etretinate mefenamic acid phentermine
fenfluramine meglumine and sodium phenylephrine
fenoprofen melphalan phenylmercuric acetate
flecainide meperidine phenylmercuric nitrate
floxuridine meprednisone pindolol
fluorometholone mercuric oxide piroxicam
fluorouracil methacycline poliovirus vaccine
flurazepam methazolamide potassium penicillin G
flurbiprofen methocarbamol potassium penicillin V
framycetin methotrexate potassium phenethicillin
gold Au 198 methoxsalen practolol
gold sodium thiomalate methylergonovine prazepam
gold sodium thiosulfate methylprednisolone prazosin
halazepam methysergide prednisolone
hexachlorophene metocurine iodide prednisone
hydrabamine penicillin metoprolol procaine penicillin G
V metronidazole procarbazine
hydralazine mexiletine propranolol
hydrocortisone mianserin protriptyline
hydroxyurea midazolam pyrilamine
ibuprofen minocycline rabies immune globulin
imipramine minoxidil rabies vaccine
influenza virus vaccine mitomycin ranitidine
insulin mitotane rifampin
iodipamide meglumine moxalactam rubella and mumps virus
iothalamate meglumine mumps virus vaccine vaccine (live)
and sodium (live) semustine
iothalamic acid nadolol smallpox vaccine
iron dextran nalorphine spironolactone
isotretinoin naloxone streptomycin
ketoprofen naltrexone streptozocin
labetalol naproxen succinylcholine
levallorphan neomycin sulindac
temazepam timolol trioxsalen
tetanus immune globulin tocainide tripelennamine
tetanus toxoid trazodone triprolidine
tetracycline triazolam tubocurarine
thimerosal triethylenemelamine uracil mustard
thiotepa trimipramine verapamil
McCulley JP, et al. Classification of chronic blepharitis. Ophthalmology 1982; 89:1173.
Yeatts RP, White WL. Granulomatous blepharitis as a sign of Melkersson-Rosenthal

syndrome. Ophthalmology 1997; 104: 1185-1190.
ACUTE BLEPHARITIS (INFLAMMATION OF LIDS WITH RAPID ONSET)
1. Usual allergy to drugs, including the following:

acenocoumarin ampicillin bleomycin
acetaminophen amyl nitrite bromide
acetanilid anisindione bupivacaine
acetazolamide anisotropine busulfan
acetohexamide antazoline butabarbital
acetophenazine antipyrine butacaine
acetyldigitoxin aprobarbital butalbital
actinomycin C aspirin butallylonal
acyclovir atropine butaperazine
adenine arabinoside atropine methylnitrate butethal
adiphenine auranofin cactinomycin
allobarbital aurothioglucose carbachol
allopurinol aurothioglycanide carbamazepine
alprazolam bacitracin carbenicillin
aluminum nicotinate barbital belladonna carbimazole
ambutonium(?) bendroflumethiazide carisoprodol
aminopterin benoxinate carmustine
aminosalicylate(?) benzalkonium carphenazine
aminosalicylic acid(?) benzathine penicillin G cefaclor
amithiozone benzphetamine cefadroxil
amobarbital benzthiazide cefamandole
amodiaquine betamethasone cefazolin
amoxicillin betaxolol cefonicid
amphotericin B bishydroxycoumarin cefoperazone
ceforanide cytarabine ergotamine
cefotaxime dacarbazine erythromycin
cefotetan dactinomycin ethionamide
cefoxitin daunorubicin ethopropazine
cefsulodin deferoxamine ethosuximide
ceftazidime demecarium ethotoin
ceftizoxime deslanoside ethoxzolamide
ceftriaxone dexamethasone ethyl biscoumacetate
cefuroxime diatrizoate meglumine and etidocaine
cephalexin sodium F3T
cephaloglycin diazepam fenfluramine
cephaloridine diazoxide fluorescein
cephalothin dibucaine fluorometholone
cephapirin dichlorphenamide fluorouracil
cephradine dicloxacillin fluoxymesterone
chloral hydrate dicumarol fluphenazine
chlorambucil dicyclomine flurazepam
chloramphenicol diethazine framycetin
chlordiazepoxide diethylcarbamazine furosemide
chloroprocaine diethylpropion gentamicin
chloroquine digitalis gitalin
chlorothiazide digitoxin glutethimide
chlorphentermine digoxin glyburide
chlorpromazine dimercaprol glycopyrrolate
chlorpropamide dimethyl sulfoxide gold Au 198
chlorprothixene diphemanil gold sodium thiomalate
chlortetracycline diphenadione gold sodium thiosulfate
chlorthalidone diphenylhydantoin griseofulvin
chrysarobin diphtheria and tetanus halazepam
clidinium toxoids (adsorbed) haloperidol
clindamycin diphtheria and tetanus heparin
clomiphene toxoids and pertussis heptabarbital
clonazepam diphtheria and tetanus hetacillin
clorazepate vaccine (adsorbed) hexethal
cloxacillin diphtheria toxoid adsorbed hexobarbital
cobalt dipivefrin hexocyclium
cocame disulfiram homatropine
colistin doxorubicin hyaluronidase
colloidal silver dromostanolone hydrabamine penicillin
cortisone droperidol V
cyclobarbital dyclonine hydralazine
cyclopentobarbital echothiophate hydrochlorothiazide
cyclopentolate edrophonium emetine hydrocortisone
cyclophosphamide ephedrine hydroflumethiazide
cycloserine epinephrine hydromorphone
cyclothiazide ergonovine hydroxyamphetamine
hydroxychloroquine methicillin oxyphencyclimine
idoxuridine methimazole oxyphenonium
indapamide methitural paramethadione
influenza virus vaccine methixene penicillin
insulin methohexital pentobarbital
iodide and iodine solutions methotrexate perazine
and compounds methotrimeprazine pericyazine
iodipamide meglumine methsuximide perphenazine
iothalamate meglumine methyclothiazide phenacaine
and sodium methylatropine nitrate phenacetin
iothalamic acid methyldopa phendimetrazine
isoflurophate methylergonovine phenindione
isoniazid methylprednisolone phenobarbital
isopropamide methylthiouracil phenprocoumon
kanamycin methyprylone phensuximide
lanatoside C methysergide phentermine
levallorphan metolazone phenylbutazone
levobunolol metrizamide phenylephrine
levodopa midazolam phenylmercuric acetate
lidocaine mild silver protein phenylmercuric nitrate
lincomycin mitomycin phenytoin
lomustine morphine physostigmine
lorazepam moxalactam pilocarpine
measles and rubella virus mumps virus vaccine (live) pipenzolate
vaccine (live) nafcillin piperacetazine
measles virus vaccine nalorphine piperazine
(live) naloxone piperidolate
measles, mumps, and naltrexone piperocaine
rubella virus vaccine naphazoline pipobroman
(live) naproxen poldine
mechlorethamine neomycin poly thiazide
medrysone neostigmine polymyxin B
melphalan niacinamide potassium penicillin G
mepenzolate nicotinic acid potassium penicillin V
meperidine nicotinyl alcohol potassium phenethicillin
mephenytoin nitrazepam potassium
mephobarbital nitrofurantoin phenoxymethyl
mepivacaine nitromersol prazepam
meprobamate nystatin prednisolone
mercuric oxide opium prilocaine
mesoridazine oral contraceptives primidone
methacholine ouabain probarbital
methantheline oxacillin procaine
metharbital oxprenolol procaine penicillin G
methazolamide oxymorphone prochlorperazine
methdilazine oxyphenbutazone promazine
promethazine sulfamerazine thiopropazate
propantheline sulfameter thioproperazine
proparacaine sulfamethazine thioridazine
propiomazine sulfamethizole thiotepa
propoxycaine sulfamethoxazole thiothixene
propranolol sulfamethoxypyridazine timolol
propylthiouracil sulfanilamide tolazamide
quinethazone sulfaphenazole tolbutamide
quinidine sulfapyridine trazodone
quinine sulfasalazine triazolam
rabies immune globulin sulfathiazole trichlormethiazide
rabies vaccine sulfisoxazole tridihexethyl
radioactive iodides talbutal triethylenemelamine
scopolamine temazepam trifluoperazine
secobarbital testolactone trifluorothymidine
semustine testosterone trifluperidol
silver nitrate tetanus immune globulin triflupromazine
silver protein tetanus toxoid trifluridine
sodium salicylate tetracaine trimeprazine
streptomycin tetracycline trimethadione
streptozocin tetrahydrozoline tropic amide
succinylcholine thiabendazole uracil mustard
sulfacetamide thiamylal vancomycin
sulfachlorpyridazine thiethylperazine vidarabine
sulfadiazine thimerosal vinbarbital
sulfadimethoxine thiopental warfarin
*2. Infections, such as bacterial, fungal, and viral
Tasman W, Jaeger E, eds. Duane's clinical ophthalmology. Philadelphia: JB Lippincott,

1990.
THICKENED EYELIDS
1. Trachoma
2. Multiple chalazia
*3. Chronic conjunctivitis
*4. Blepharitis-lid margins thickened
5. Tarsitis-rare, such as in syphilis or tuberculosis
6. Trisomy (E syndrome)
7. Congenital hypothyroidism
8. Pheochromocytoma, medullary thyroid carcinoma, and neurofibromatosis
Baum JL, Adler ME. Pheochromocytoma, medullary thyroid carcinoma, and multiple
mucosal neuroma. Arch Ophthalmol 1972; 87:574-584.
Gellis SS, Feingold M. Atlas of mental retardation. Washington, DC: US Government

Printing Office, 1968.
BLEPHAROPHIMOSIS (SHORT PALPEBRAL FISSURE)
1. Blepharochalasis
2. Blepharofacioskeletal syndrome
3. Blepharophimosis-amenorrhea syndrome (blepharophimosis, ptosis, epicanthus
inversus syndrome)
5. Clefting syndrome with anterior chamber and lid anomalies
6. Craniocarpotarsal syndrome (Freeman-Sheldon syndrome; whistling face syndrome)
7. Down syndrome (trisomy 21, mongolism)
8. Dubowitz syndrome
9. 18P syndrome
10. Kaufman oculocerebrofacial syndrome
11. Klein-Waardenburg syndrome
12. Komoto syndrome (congenital eyelid tetrad)
13. Marden-Walker syndrome
14. Meyer-Schwickerath and Weyers syndrome
15. Michel syndrome
*16. Microphthalmos
17. Mohr syndrome (orofaciodigital syndrome II)
18. Mohr syndrome
19. Oculopalatoskeletal syndrome
20. Ohdo blepharophimosis syndrome
21. Pena-Shokeir type II syndrome
22. Progeria (Hutchinson-Gilford syndrome)
24. Ring chromosome in the D group (13-15) (ring D syndrome)
25. Schonenberg syndrome (dwarf cardiopathy syndrome)
26. Schwartz-Jampel syndrome (osteochondromuscular dystrophy)
27. Simosa syndrome
28. Syndrome of blepharophimosis with myopathy
*29. Traumatic
30. Trisomy (E syndrome) (Edward syndrome)
32. Young-Simpson syndrome
33. X-linked mental retardation syndrome
34. 3p- syndrome
35. 10q- syndrome
Bonthron DT, et al. Parental consanguinity in the blepharophimosis, heart defect,
hypothyroidism, mental retardation syndrome (Young-Simpson syndrome). J Med Genet
1993; 30:255-256.
Melnyk AR. Blepharophimosis, ptosis, and mental retardation: further delineation of

Ohdo syndrome. Clin Dysmorphol 1994; 3:121-124.
Suri M, et al. Blepharophimosis, telecanthus, microstomia, and unusual ear anomaly

Simosa syndrome in an infant. Am J Med Genet 1994; 51:222-223.
EURYBLEPHARON
Euryblepharon is defined as a horizontally elongated palpebral aperture (normal,

30 mm) and may be associated with ectropion and present in other family members.
1. Excessive tension of skin

2. Defective separation of the lids
3. Excessive pull of the platysma
*4. Localized displacement of the lateral canthi
5. Hypoplasia of tarsus
Feldman E, et al. Euryblepharon: a case report with photographs documenting the

condition from infancy to adulthood. J Pediatr Ophthalmol Strabismus 1980; 17:307-
309.
Gupta AK, et al. Euryblepharon. J Pediatr Ophthalmol 1972; 9:173-174.
McCord CD, et al. Congenital euryblepharon. Ann Ophthalmol 1979; 11:1217-1224.
LID COLOBOMA
1. Amniogenic band syndrome (amniotic bands-Streeter anomaly)

2. Epidermal nevus syndrome
3. Facial c1efting syndrome (Tessier syndromes)
4. Fraser syndrome
5. Frontonasal dysplasia syndrome
7. Miller syndrome
8. Nager syndrome
9. Nevus sebaceous of Jadassohn (linear sebaceous nevus syndrome)
10. Palpebral coloboma-lipoma syndrome
*11. Traumatic
12. Treacher Collins-Franceschetti syndrome (mandibulofacial dysostosis)
Braude LL, et al. Ocular abnormalities in the amniogenic band syndrome. Br J
Ophthalmol 1981; 65:299-303.
Burch JV, et al. Ichthyosis hystrix (epidermal nevus syndrome) and Coats' disease. Am J
Opthalmol 1980; 89:25-30.
Isenberg SJ. The eye of infancy. Chicago: Year Book Medical, 1989.
NECROSIS OF EYELIDS

acenocoumarol diphenadione phenprocoumon
amphotericin B ethyl biscoumacetate tobramycin
anisindione nafcillin warfarin
dicumarol phenindione
*2. Mechanical, electrical, or thermal trauma

3. Periorbital cellulitis-periorbital necrotizing cellulitis
4. Secondary to infection
Scott PM, Bloome MA. Lid necrosis secondary to streptococcal periorbital cellulitis. Ann
POLIOSIS (WHITENING OF HAIR, EYEBROWS, AND EYELASHES)
1. Albino
2. Alopecia areata
*3. Aging
amodiaquine dexamethasone medrysone
betamethasone epinephrine prednisolone
chloroquine fluorometholone thiotepa
cortisone hydrocortisone
cyclosporin A hydroxychloroquine

6. Radiation therapy
7. Rubinstein-Taybi syndrome
8. Severe dermatitis
9. Stress
*10. Unknown etiology
11. Vitiligo
12. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis syndrome)
14. Werner syndrome (progeria of adults)
Rosner F. Can hair turn white overnight? JAMA 1981; 246:2324.
Wilkins, 1985.
TRICHOMEGALY (LONG LASHES)
1. Associated with cataract and hereditary spherocytosis

2. Congenital with pigmentary retinal degeneration, dwarfism, and mental retardation
3. Cyclosporine induced
4. De Lange syndrome (congenital muscular hypertrophy-cerebral syndrome)
5. Ectodermal dysplasia (Curtius syndrome)
6. Human immunodeficiency virus (HIV)
7. Hypertrichosis (hirsutism)
8. Isolated adrenal malfunction and ovarian atrophy
*10. Normal
11. Oliver-McFarlane syndrome
13. Schwartz syndrome
Chang TS, et al. Congenital trichomegaly, pigmentary degeneration of the retina and
growth retardation Oliver-McFarlane syndrome year follow-up of the first reported case.
Can J Ophthal 1993; 28:191-193.
MADAROSIS (LOSS OF EYELASHES)
1. Chronic skin diseases, including psoriasis, neurodermatitis, exfoliative dermatitis,

ichthyosis, alopecia areata, acne, lichen planus, epidermolysis bullosa, lupus
erythematosus, acanthosis nigricans, dermatophytosis, hereditary ectodermal dysplasia
syndrome and acrodermatitis
2. Congenital atrichia
3. Cryptophthalmos
4. Cutaneous T cell lymphoma
5. Demodicosis
acebutolol(?) bismuth sodium dichlorphenamide(?)

acenocoumarin(?) triglycollamate(?) dicumarol(?)
acetazolamide(?) bismuth subcarbonate(?) diethylcarbamazine
acetohexamide(?) bismuth subsalicylate(?) diethylpropion(?)
acid bismuth sodium bleomycin(?) diltiazem(?)
tartrate broxyquinoline(?) diphenadione(?)
actinomycin C(?) busulfan(?) divalproex sodium(?)
alcohol(?) cactinomycin doxepin(?)
allopurinol(?) captopril(?) doxorubicin
aluminum nicotinate carbamazepine(?) dromostanolone(?)
amantadine(?) carbimazole(?) droperidol(?)
aminopterin(?) carmustine enalapril(?)
aminosalicylate(?) CCNU epinephrine
aminosalicylic acid(?) chlorambucil(?) ergonovine(?)
amiodarone(?) chloroquine(?) ergotamine(?)
amithiozone(?) chlorphenterrnine(?) ethionamide(?)
amitriptyline(?) chlorpropamide(?) ethotoin(?)
amodiaquine(?) cimetidine(?) ethoxzolamide(?)
amoxapine(?) cisplatin ethyl biscoumacetate(?)
amphetamine(?) clofibrate(?) etretinate(?)
anisindione(?) clomiphene(?) fenfluramine(?)
aspirin(?) clonazepam(?) fenoprofen(?)
atenolol(?) colchicine(?) flecainide(?)
auranofin(?) cyclophosphamide(?) floxuridine(?)
aurothioglucose(?) cytarabine(?) fluorouracil(?)
aurothioglycanide(?) dacarbazine fluoxymesterone(?)
azathioprine(?) dactinomycin(?) gentamicin
benzphetamine(?) danazol(?) glyburide
betaxolol daunorubicin glycopyrrolate(?)
bishydroxycoumarin(?) desipramine(?) gold au 198
bismuth oxychloride(?) dextroamphetamine(?) gold sodium
bismuth sodium tartrate(?) dextrothyroxine(?) thiomalate(?)
bismuth sodium diacetylmorphine(?) gold sodium
thioglycollate(?) DIC thiosulfate(?)
guanethidine(?) morphine(?) sulfadiazine(?)
haloperidol(?) nadolol(?) sulfadimethoxine(?)
HCNU naltrexone(?) sulfamerazine(?)
heparin(?) naproxen(?) sulfameter(?)
hydroxychloroquine(?) niacin(?) sulfamethazine(?)
hydroxyurea(?) niacinamide(?) sulfamethizole(?)
ibuprofen(?) nicotinyl alcohol(?) sulfamethoxazole
imipramine(?) nifedipine(?) sulfamethoxypyridazine(
indomethacin(?) nitrofurantoin(?) ?)
interferon(?) nortriptyline(?) sulfanilamide(?)
iodochlorhydroxyquin(?) opium(?) sulfaphenazole(?)
iodoquinol(?) oral contraceptives(?) sulfapyridine(?)
isotretinoin(?) oxprenolol(?) sulfasalazine(?)
ketoprofen(?) paramethadione(?) sulfathiazole(?)
labetalol(?) penicillamine(?) sulfisoxazole
levobunolol phendimetrazine(?) sulindac(?)
levodopa(?) phenindione(?) tamoxifen(?)
lithium carbonate(?) phenmetrazine(?) testolactone(?)
lomustine phenprocoumon(?) testosterone(?)
maprotiline(?) phentermine(?) tetracycline(?)
mechlorethamine(?) pindolol(?) thiotepa
melphalan(?) pipobroman(?) timolol maleate
mephenytoin(?) piroxicam tocainide(?)
methamphetamine(?) prazosin(?) tolazamide(?)
methazolamide(?) procarbazine(?) tolbutamide(?)
methimazole(?) propranolol(?) triethylenemelamine(?)
methotrexate(?) propylthiouracil(?) trifluperidol(?)
methylergonovine(?) protriptyline(?) trimethadione(?)
methylthiouracil(?) pyridostigmine(?) uracil mustard(?)
methysergide(?) ranitidine valproate sodium(?)
metoprolol(?) semustine valproic acid(?)
mexiletine(?) sodium salicylate(?) verapamil(?)
mianserin(?) streptomycin(?) vinblastine(?)
minocycline(?) streptozocin vincristine(?)
minoxidil(?) sulfacetamide(?) vitamin A
mitomycin sulfachlorpyridazine(?) warfarin(?)
mitotane(?) sulfacytine(?)
7. Ehlers-Danlos syndrome, unspecified type

*8. Endocrine disease, including hypothyroidism, hyperthyroidism, pituitary
insufficiency, hypoparathyroidism, and pituitary necrosis syndrome (Simmonds-Sheehan
syndrome)
9. Following eyelid tattooing
10. Generalized hypotrichosis
11. HIV (human immunodeficiency virus)
12. Hypocalcemia
13. Hypothermal injury
*14. Idiopathic
15. Inflammation and infection of the lids, including seborrheic blepharitis, squamous
blepharitis, herpes zoster, sebaceous gland carcinoma, vaccinia, mycotic infection,
furuncles, and erysipelas
16. Intoxication with arsenic, bismuth, thallium, gold, quinine and vitamin A
17. Isolated madarosis
18. Keratosis decalvans
19. Keratosis follicularis
20. Keratosis spinulosa
21. Lid colobomas
22. Leprosy
23. Lipoid proteinosis (Urbach-Wiethe syndrome)
24. Polymorphous light eruption
25. Pseudoprogeria syndrome
26. Radiation
27. Severe debilitating systemic diseases, including tuberculosis, syphilis, sickle cell
anemia, cholera, and Hansen disease (leprosy)
28. Trauma
29. Vogt-Koyanagi-Harada disease (uveitis-vitiligo-alopecia-poliosis syndrome)
Extracted Table Madarosis (loss of eyelashes)
Dana MR, et al. Ocular manifestations of leprosy in a noninstitutionalized community in

the United States. Arch Ophthalmol 1994; 112:626-629.
Butterworth-Heinemann, 200l.
DISTICHIASIS (ACCESSORY ROW OF LASHES GROWING FROM

OPENINGS OF MEIBOMIAN GLAND)
1. Acquired
A. Chemical
B. Immunologic
C. Physical
*2. Congenital
A. Anodontia-hypotrichosis syndrome
B. Distichiasis, lymphedema syndrome
C. Ectropion and distichiasis
D. Idiopathic eyelid edema
E. Pierre Robin syndrome
F. Tristichiasis
3. Hereditary-autosomal dominant
Fraunfelder FT, Roy FH. Current ocular therapy, 5th ed. Philadelphia: WB Saunders,
2000.
Kolin T, et al. Hereditary lymphedema and distichiasis. Arch Ophthalmol 1991; 109:980-
982.
Temple IK, Collin JR. Distichiasis-lymphoedema syndrome: a family report. Clin

Dysmorphol 1994; 3:139-142.
COARSE EYEBROWS
1. Congenital hypothyroidism (cretinism)
2. CPD syndrome (chorioretinopathy and pituitary dysfunction)
3. Hunter syndrome [mucopolysaccharidosis (MPS II)]
4. Hurler syndrome (MPS I)
*5. Normal variation
7. Sanfilippo syndrome (MPS III)
SYNOPHRYS (CONFLUENT EYEBROWS EXTENDING TO MIDLINE)
1. Basal cell nevus syndrome (Gorlin syndrome)

2. Cornelia De Lange syndrome (congenital muscular hypertrophy-cerebral syndrome)
3. Deletion 3p syndrome
4. Duplication 3q syndrome
5. Frontometaphyseal dysplasia
6. Hirschhorn-Cooper syndrome (chromosome partial deletion syndrome)
7. Labard syndrome
*8. Normal variation
9. Partial trisomy chromosome 15
11. Thirteen trisomy syndrome (Patau syndrome)
12. Waardenburg syndrome (interoculoiridodermatoauditive dysplasia)
Smith DW. Recognizable patterns of human malformation. Philadelphia: WB Saunders,
1970.
HERTOGH SIGN (LACK OF OUTER THIRD OF EYEBROWS)
1. Autonomic nervous system dysfunction

2. Diphtheria
*3. Endocrinopathies
4. Hypogonadism
5. Hypothyroidism
6. Neurodermatitis
7. Scleroderma (systemic scleroderma)
Pau H. Differential diagnoses of eye diseases, 2nd ed. New York: Thieme Medical, 1988.
LID MYOKYMIA (SPONTANEOUS FASCICULAR EYELID TREMOR

WITHOUT MUSCULAR ATROPHY OR WEAKNESS)
*1. Not associated with organic disease

A. Fatigue
B. Lack of sleep
C. Bright light dazzle
D. Irritative corneal or conjunctival lesions
E. Debility or anemia
F. Excessive alcohol or smoking
G. Overwork
2. Followed by spastic paretic facial contracture-in dorsal pons in adult and children
4. Trigeminal neuralgia
5. Myasthenia gravis (pseudoparalytic syndrome)
6. Familial occurrence
7. Autosomal dominant familial dystonia
2000.
PRESEPTAL CELLULITIS OF EYELID
1. Eczema
*2. Hordeolum
3. Neonatal conjunctivitis
4. Otitis media
5. Sinusitis
6. S. aureus
7. Toxic shock syndrome
8. Trauma
9. Upper respiratory tract
10. Varicella
Brower MF, et al. Preseptal cellulitis complicated by toxic shock syndrome. Arch
Ophthalmol 1987; 105: 1631-1632.
Weiss A, et al. Bacterial periorbital and orbital cellulitis in childhood. Ophthalmology

1983; 90:195- 203.
TELECANTHUS (DISPROPORTIONATE INCREASE IN DISTANCE

BETWEEN MEDIAL CANTID; MEASUREMENTS IN INFANTS ARE 18 TO 22
MM)
*1. Primary
2. Secondary-may occur secondarily to an increased distance between the bony orbits
(see hypertelorism)
A. Aarskog syndrome
B. Blepharonasofacial syndrome
C. Blepharophimosis syndrome
D. Camptomelic dysplasia
E. Carpenter syndrome
F. Cerebrofacioarticular syndrome of van Maldegem
G. Coffin-Lowry syndrome
H. de Lange syndrome
I. Deletion 5g syndrome
J. Dubowitz syndrome
K. Facial-renal acromesomelic syndrome
L. Faciooculoacousticorenal syndrome
M. Fetal alcohol syndrome
N. Fetal hydantoin syndrome
O. Frontonasal dysplasia
P. Lambotte syndrome
Q. KBG syndrome (initials of family studied)
R. Michel syndrome
S. Nasopalpebral lipoma-coloboma syndrome
T. Oculodentodigital syndrome
U. Orofaciodigital (OFD) type I and type II (Mohr syndrome)
V. Prader-Willi syndrome
W. Rieger syndrome
X. Simosa syndrome
Y. Tetra-X syndrome
Z. Trisomy syndrome
AA. Toriello-Carey syndrome
BB. Trauma
CC. Waardenburg syndrome
DD. Williams syndrome
EE. 5p- syndrome (Cri-du-chat)
FF. 10q- syndrome
GG. Fetal hydantoin syndrome
Suri M, et al. Blepharophimosis, telecanthus, microstomia, and unusual ear anomaly

(Simosa syndrome) in an infant. Am J Med Genet 1994; 51:222-223.

1990.
ANKYLOBLEPHARON (PARTIAL OR COMPLETE FUSION OF UPPER TO

LOWER EYELIDS)
*1. Ablepharon, macrostomia syndrome

2. Ankyloblepharon ectodermal dysplasia, cleft lip and palate
3. Curly hair, ankyloblepharon, nail dysplasia syndrome (CHANDS)
4. Cryptophthalmos (complete fusion of lids)
5. Diphtheritic conjunctivitis
6. Ectodermal syndrome
7. Edward syndrome
8. Fraser syndrome
9. Gastrointestinal anomalies
10. Hay-Wells ectodermal pterygium syndrome
11. Popliteal pterygium syndrome
12. Smallpox
13. Trachoma
14. Trisomy 18
15. Ulcerative blepharitis
Roy FH. Ophthalmic surgery: approaches by the masters. Philadelphia: Lea & Febiger,
1995.
FLARING OF NASAL PART OF EYEBROW

1. Blepharonasofacial syndrome
2. Partial trisomy 10q syndrome
*3. Waardenburg syndrome
HIGH ARCHED BROW
1. Kabuki makeup syndrome

2. Shprintzen-Goldberg syndrome
ABSENT BROW HAIR
1. Cryptophthalmos
2. Duplication 14q syndrome
4. Pseudoprogeria syndrome
TRICHIASIS (INWARD TURNING LASHES)
*1. Inflammation/infection
A. Chronic blepharitis
B. Herpes simplex or zoster
C. Trachoma
2. Lid Tumors
A. Basal cell carcinoma
B. Capillary hemangioma
C. Conjunctiva amyloidosis
3. Medications
A. Epinephrine
B. Idoxuridine
C. Phospholine iodide
D. Pilocarpine
E. Practolol
F. Trifluridine
G. Vidarabine
4. Systemic/Immunologic Disorders
A. Erythema multiforme
B. Ocular cicatricial pemphigoid
C. Stevens-Johnson syndrome
D. Toxic epidermal necrolysis
E. Vernal kertoconjunctivitis
5. Trauma
A. Chemical injury (lye)
B. Mechanical injury or repair of injury
(1) Lower lid transconjunctival approach for floor fracture repair or
blepharoplasty
(2) After enucleation
(3) After ectropion repair
(4) Thermal bums to face/lids
C. Surgery
Byrnes GA. Congenital distichiasis. Arch Ophthalmol 199l; 109:l752-l753.
Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc 1986; 84:527-663.
Roy FH. Ophthalmic surgery: approaches by the masters. Philadelphia: Lea & Febiger,
1995.
Udell IJ. Trifluridine-associated conjunctival cicatrization. Am J Ophthalmol 1976;

82:117-l21.
3
Lacrimal System
CONTENTS
Dacryoadenitis 110
Acute 110
Chronic 110
Diagnostic table 112
Mikulicz syndrome-Diagnostic table 113
Painless enlargement of lacrimal gland 111
Painful enlargement of lacrimal gland 111
Bloody tears 111
Excessive tears 115
Hypersecretion of tears 115
Inadequacy of lacrimal drainage system 118
Drugs found in tears 122
Dry eye 122
Dacryocystitis 125
Acute 125
Chronic 125
DACRYOADENITIS (INFLAMMATION OF LACRIMAL GLAND)
1. Acute dacryoadenitis-rare catarrhal inflammation of the lacrimal gland that usually

accompanies systemic disease
A. In children-*mumps, measles, influenza, scarlet fever, erysipelas, typhoid
fever
B. In adults-gonorrhea, endogenous conjunctivitis and uveitis, infectious
mononucleosis, typhoid fever, Crohn disease
C. Secondary to inflammation from lids or conjunctiva, to include Klebsiella
pneumoniae, coliform organisms, *Staphylococcus, *Streptococcus, Aedes
aegypti, *Diplococcus pneumoniae, and *Neisseria gonorrhea
2. Chronic dacryoadenitis-proliferative inflammation of the lacrimal gland, usually
because of specific granulomatous disease
A. Boeck sarcoid (Schaumann syndrome)
B. Heerfordt disease-chronic bilateral parotitis and uveitis, often associated with
paresis of the cranial nerves, usually the seventh nerve, and other general
symptoms
*(1) Sarcoidosis syndrome (Schaumann syndrome)
(2) Tuberculosis
C. Mikulicz syndrome-dacryoadenitis and parotitis manifested by chronic
bilateral swelling of the lacrimal and salivary glands
(1) Bang disease (brucellosis)
(2) Hodgkin disease
(3) Leukemia
(4) Lymphoma
(5) Lymphosarcoma (Brill-Symmers disease)
(6) Reticuloendothelial disease
(7) Mumps
*(8) Sarcoidosis syndrome (Schaumann syndrome)
(9) Syphilis
(10) Tuberculosis
(11) Waldenstrom macroglobulinemia
D. Miliary tuberculosis
E. Pseudotumor
F. Syphilis (gumma)
3. Painless enlargement of lacrimal gland
A. Leukemia
B. Mumps
4. Painful enlargement of lacrimal gland
A. Autoimmunologically mediated syndrome
B. Lymphomatous disease (25%)
C. Chronic enlargement arising from sarcoid or orbital pseudotumor (25%)
D. Lacrimal gland neoplasm (50%)
(1) Benign
a. Adenoma
b. Mixed tumor
(2) Malignant
a. Carcinoma unrelated to mixed tumor
b. Adenocarcinoma (adenoid cystic carcinoma)
c. Mucoepidermoid carcinoma
d. Squamous cell carcinoma
e. Mixed tumor
Divine RD, et al. Metastatic carcinoid unresponsive radiation therapy presenting as a

lacrimal fossa mass. Ophthalmology 1982; 89:516.
Newell FW. Ophthalmology, principles and concepts, 7th ed. St. Louis: CV Mosby,
1992.
Rhem MN, et al. Epstein-Barr virus dacryoadenitis. Am J Ophthalmol 2000; 129:372-

375.
Roy FH. Ocular syndrome and systemic diseases, 3rd ed. Philadelphia: Lippincott
Shields CL, et al. Clinicopathologic review of cases of lacrimal gland lesions.

BLOODY TEARS
1. Conjunctiva
A. Application of a drug such as silver nitrate
B. Cachectic conjunctivitis
C. Focal dermal hypoplasia syndrome (Goltz syndrome)
D. Fibroma
*E. Giant papillary conjunctivitis secondary to contact lens wear or prosthesis
wear
F. Gross disturbance of autonomic nervous system
G. Hemangioma
H. Hereditary hemorrhagic telangiectasis
I. Inflammatory granuloma
J. Malignant melanoma
K. Metastatic carcinoid tumor
*L. Severe conjunctivitis with marked hyperemia
*M. Subconjunctival hemorrhage following sudden venous congestion of head
from stooping, coughing, choking, Valsalva trauma, hemophilia or advanced
athrombia
N. Vicarious menstruation with ectopic tissue
2. Corneal vascular lesion or pannus
3. Lid
A. Pubic lice and nits on the lashes
B. Trauma
4. Other
A. Familial telangiectasis
B. Hemophilia
C. Hysteria
D. Jaundice
E. Osler-Weber-Render
F. Pathologic process of lacrimal gland
G. Severe anemia
H. Severe epistaxis with regurgitation through the lacrimal passages
Dun S, et al. Acute dacryoadenitis and Crohn's disease: findings and management.
Ophthalmic Plastic & Reconstructive Surg 1992; 8:295-299.
Gritz DC, Rao NA. Metastatic carcinoid tumor diagnosis from a caruncular mass. Am J
Ophthalmol 199l; 112: 468-469.
Hornblass A, et al. The management of epithelial tumors of the lacrimal sac.

Krohel GB, et al. Bloody tears associated with familial telangiectasis. Arch Ophthalmol
1987; 105:1489-l490.
Extracted Table Acute dacryoadenitis (inflammation of lacrimal gland)
Extracted Table Mikuliez syndrome- chronic dacryoadenitis and parotitis
Extracted Table Bloody Tears
EXCESSIVE TEARS
1. Hypersecretion of tears-may be due to basic secretors (mucin, lacrimal, including

secretion from glands of Kraus and Wolfring and oil, including secretion from Zeis, Moll,
and Meibomian palpebral glands) or reflex secretors (main lacrimal glands and accessory
palpebral glands)
A. Primary (disturbance of lacrimal gland)
B. Central or psychic
(1) Central nervous system lues
(2) Corticomeningeal lesions
(3) Emotional states
(4) Hysteria
(5) Physical pain
(6) Voluntary lacrimation, such as when acting
C. Neurogenic
(1) Ametropia, tropia, phoria, and eyestrain or fatigue
(2) Caloric, lacrimal, and reflex tearing-bilateral lacrimation when
syringing the ear with warm or cold water and during Tensilon testing
(3) Crocodile or alligator tears-unilateral profuse tearing when eating
a. Congenital, often associated with ipsilateral paresis of lateral
rectus muscle
b. Acquired with onset in early stage of facial palsy (Bell palsy) or
sequela with parasympathetic fibers to the otic ganglion growing
back into superficial petrosal nerve
c. Duane retraction syndrome
(4) Bell palsy (idiopathic facial paralysis)
(5) Marin-Amat syndrome (inverted Marcus Gunn Jaw-wink
phenomenon)
(6) Melkersson-Rosenthal syndrome (Melkersson idiopathic fibro edema)
d. Section of the greater superficial petrosal nerve
acebutolol atenolol chlordiazepoxide
acetophenazine beclomethasone chlorpromazine
acetylcholine betamethasone ciprofloxacin
adrenal cortex injection bethanechol clonazepam
alcohol bishydroxycoumarin clorazepate
aldosterone bleomycin codeine
alseroxylon butaperazine dactinomycin
ambenonium carbachol dantrolene
antazoline carphenazine desoxycorticosterone
Apresoline chloral hydrate dexamethasone
dextran lithium carbonate prazepam
diazepam lorazepam prochlorperazine
diazoxide meprednisone promazine
dicumarol mesoridazine promethazine
diethazine methacholine propiomazine
diethylcarbamazine methaqualone propoxyphene
diltiazem methdilazine propranolol
disodium pamidronate methotrimeprazine pyridostigmine
doxorubicin methylprednisolone pyridostigmine
edrophonium metoprolol quinidine
epinephrine midazolam pyrilamine
ether mitomycin quinidine
ethopropazine morphine rauwolfia serpentina
etretinate nadolol naltrexone rescinnarnine
floxuridine nalorphine reserpine
fludrocortisone naloxone rifampin
fluorometholone neostigmine syrosingopine
fluorouracil nifedipine temazepam
fluphenazine nitrazepam thiethylperazine
fluprednisolone opium thiopropazate
flurazepam oxazepam thioproperazine
glycerin paramethasone thioridazine
halazepam pentazocine triazolam
heparin perazine trichloroethylene
hydralazine pericyazine trifluoperazine
hydrocortisone perphenazine triflupromazine
hydroxyamphetamine phenylephrine trimeprazine
indomethacin(?) pindolol tripelennamine
isotretinoin piperacetazine verapamil
ketarnine piperazine vinblastine
labetalol piroxicam warfarin
levallorphan practolol
(8) Exposure to wind, cold, or bright light; photosensitivity and sunburn

(9) Glaucoma
(10) Homer syndrome (see p. 59) (cervical sympathetic paralysis
syndrome)
(11) Inflammation or infection of the conjunctiva, uvea, cornea, orbit, lids,
sinuses, teeth, or ears
a. Acute hemorrhagic conjunctivitis
b. Avitaminosis B (pellagra, niacin deficiency)
c. Conjunctivochalasis
d. Elschnig syndrome (I) (meibomian conjunctivitis)
e. Epidemic keratoconjunctivitis
f. Feer syndrome (acrodynia)
g. Hanhart syndrome (recessive keratosis palmoplantaris)
h. Keratodermia palmaris et plantaris
i. Reiter syndrome (polyarthritis enteric)
j. Stannus cerebellar syndrome (riboflavin deficiency)
k. Thelaziasis
(12) Lesions affecting the lids
a. Acrodermatitis chronic atrophicans
b. Blepharoptosis
c. Congenital distichiasis
*d. Ectropion
*e. Entropion
f. Epiblepharon
g. Eyelid retraction
*h. Facial paralysis
i. Lid imbrication syndrome
j. Papilloma
k. Punctal apposition
l. Trachoma
*m. Trichiasis
(13) Morquio-Brailsford syndrome (MPS IV)
(14) Myasthenia gravis-afternoon ectropion (Erb-Goldflam syndrome)
(15) Ophthalmorhinostomatohygrosis syndrome
(16) Parkinson disease-facial akinesia
(17) Reflex, such as vomiting or laughing
(18) Sjögren syndrome (secretoinhibitor syndrome)
(19) Stimulation of some cortical areas-thalamus, hypothalamus, cervical
sympathetic ganglia, or the lacrimal nucleus
a. Diencephalic epilepsy syndrome (Penfield syndrome)
b. Encephalitis
(1) acute
(2) hemorrhagica superior
(3) lethargy
(4) periaxialis diffusa
c. Engelmann syndrome (diaphyseal dysplasia)
d. Giant-cell arteritis (temporal arteritis)
e. Hypothalamic tumors
f. Meningitis
g. Page syndrome (hypertensive diencephalic syndrome)
h. Pseudobulbar palsy from Parkinson syndrome (shaking palsy)
i. Sluder syndrome (lower facial neuralgia syndrome)
j. Tic douloureux (trigeminal neuralgia syndrome)
k. Various senile dementias
(20) Gradenigo syndrome (temporal syndrome)
(21) Raeder syndrome (paratrigeminal paralysis, cluster headache)
(22) Retroparotid space syndrome (Villaret syndrome)
(24) Rothmund syndrome (telangiectasia-pigmentation-cataract syndrome)
(25) Thermal bums
D. Symptomatic
(1) Bee sting of cornea
(2) Tabes
(3) Thyrotoxicosis (Basedow syndrome)
2. Inadequacy of lacrimal drainage system
A. Congenital anomalies of lacrimal apparatus
(1) Absence or atresia including ectrodactyly-ectodermal dysplasia-
clefting syndrome
(2) Amniotocele
*(3) Fistulas of lacrimal sac and nasolacrimal duct
(4) Lateral displacement of medial canthi with lateral displacement of
puncta and lengthening of canaliculi as in Waardenburg syndrome
(interoculoiridodermatoauditive dysplasia)
(5) Obstruction of nasolacrimal drainage system, including Walker-
Clodius syndrome (lobster claw deformity with nasolacrimal obstruction)
*(6) Unformed puncta (punctal atresia)
B. Complications from diseases such as pemphigus, Stevens-Johnson syndrome
(dermatostomatitis), and lupus.
*C. Dacryocystitis
*D. Distended canaliculi with obstruction, such as from Actinomyces israelii
(Streptothrix foersteri), papilloma, or dacryolith
E. Because of drugs, including the following:
acyclovir fluorouracil thiotepa
adenine arabinoside IDU trifluorothymide
colloidal silver indoxuridine trifluorothymidine
demecarium isoflurophate (including Fuchs-Lyell
DEP neostigmine syndrome) (allergic
echthiophate physostigmine reaction due to drugs
epinephrine quinacrine causing nasolacrimal
F3T silver nitrate obstruction)
floxuridine silver protein
*F. Eversion of inferior lacrimal punctum, including involutional ectropion

(horizontal lid laxity and retractors disinsertion)
G. Eversion of inferior lacrimal punctum secondary to ichthyosis or scleroderma
H. Goltz syndrome (focal dermal hypoplasia syndrome)
I. Inadequacy of physiologic lacrimal pump
J. Traumatic lesions of lacrimal drainage system
K. Tumor obstruction, including polyps, papillary hypertrophy, and
neurofibromas
(1) Botulinum toxin usage
(2) Dyskeratosis congenita (Zinsser-Engman-Cole syndrome)
(3) Gravity inversion
(4) Irritation from dust and gases
(5) Inflammation or destruction of turbinates
(6) Inhalation cocaine abuse
(8) Leukemia
(9) Plasmoma
(10) Rhinosporidiosis
(11) Scleroma
(12) Tuberculosis
(13) Tumors
L. Primary neoplasms
(1) Fibroma
(2) Hemangiopericytoma
(3) Melanoma
(4) Papilloma
(5) Squamous cell carcinoma
M. Secondary involvement by neoplasms
(1) Basal cell carcinoma
(2) Lethal midline granuloma
(3) Leukemia
(4) Lymphoma
(5) Maxillary sinus tumors
(6) Neurofibroma
(7) Wegener granulomatosis
N. Nasal disease
(1) Sinusitis
(2) Hypertrophic rhinitis
(3) Pseudoepiphora, such as wound fistula following intraocular operation
with leak of aqueous
Baron EM, et al. Rhabdomyosarcoma manifesting as acquired nasolacrimal duct

obstruction. Am J OphthaI1993; 115:239-242.
Friberg TR, Weinreb RN. Ocular manifestations of gravity inversion. JAMA 1985;
253:1755-1758.
Glatt HJ. Epiphora caused by blepharoptosis. Am J Ophthalmol 1991; 111:649-650.
Wojno TH. Allergic lacrimal obstruction. Am J Ophthalmol 1988; 106:48-52.
Extracted Table Excessive Tears

DRUGS FOUND IN TEARS
Drugs, including the following:

alcian blue fluorouracil sodium salicylate
amodiaquine hydroxychloroquine trypan blue
aspirin methotrexate vitamin A
chloroquine minocycline
fluorescein rose bengal
Tabbara KF, Cooper H. Minocyc1ine levels in tears of patients with active trachoma.
DRY EYE (PAUCITY OR ABSENCE OF TEARS)
1. Xerosis-local tissue changes

A. Cicatricial degeneration of conjunctiva and mucous tissues
(1) General-diphtheria
(2) Upper lid-trachoma
(3) Lower lid
a. Avitaminosis A
b. Chemical irritation (especially due to alkali)
c. Dermatitis herpetiformis (Duhring-Brocq disease)
d. Epidermolysis bullosa (Weber-Cockayne syndrome)
e. Erythema multiforme (Stevens-Johnson syndrome)
f. Ocular pemphigoid
g. Plummer-Vinson syndrome (sideropenic dysphagia syndrome)
h. Radium burns
i. Reiter syndrome (conjunctivourethrosynovial syndrome)
j. Sjögren syndrome (secretoinhibitor syndrome)
k. Uyemura syndrome (fundus albipunctatus with hemeralopia and
xerosis)
B. Exposure keratitis
(1) Anterior lamella shortage secondary to trauma or facial bum
*(2) Deficient lid closure as part of facial palsy
*(3) Ectropion (see p. 81)
(4) Eyelid retraction-Graves ophthalmopathy (incomplete blink)
(5) Exophthalmos
(6) Following botulism
*(7) Infrequent blinking, such as with progressive supranuclear palsy
(8) Lack of blinking as during coma
(9) Levator spasm
(10) Melkersson-Rosenthal syndrome (Melkersson idiopathic fibroedema)
(11) Methylmalonic aciduria
(12) Ocular proptosis
(13) Rapid evaporation in hot, dry areas
(14) Stiff, immobile, retracted lids, such as those occurring secondary to
tuberculoid leprosy (Hansen disease)
2. Keratoconjunctivitis sicca-primary tear diminution of main and accessory lacrimal
glands
A. Congenital
(1) Congenital absence of lacrimal gland as in Bonnevie-Ullrich syndrome
(2) Neurogenic
(3) Associated with generalized disturbance
a. Anhidrotic type of ectodermal dysplasia
b. Familial dysautonomia (Riley-Day syndrome)
c. Cri-du-chat syndrome (Cry of the cat syndrome)
d. Cystic fibrosis syndrome (fibrocystic disease of pancreas)
B. Neurogenic hyposecretion
(1) Central-aplasia of lacrimal nucleus or lesion of seventh nerve between
nucleus and geniculate ganglion
a. Pontine lesions
b. Basal fractures
c. Otitis media
(2) Peripheral-greater superficial petrosal nerve, sphenopalatine ganglion,
or lacrimal branch
a. Skull fractures
b. Associated with neoplasms
c. Neurologic lesion of fifth nerve (neuroparalytic keratitis)
(3) Herpes zoster of the geniculate ganglion (Ramsey-Hunt syndrome)
(4) Parasympathetic blocking drugs, such as atropine and scopolamine,
may decrease an already barely adequate secretion.
(5) Botulism
(6) Deep anesthesia
(7) Debilitating disease, such as typhus and cholera, and high temperature
(8) Allergy
C. Systemic disease
(1) Allgrove syndrome
(2) Acquired immunodeficiency syndrome (AIDS)
(3) Amyotrophic lateral sclerosis
(5) Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
acebutolol astemizole benzalkonium
acetophenazine atenolol benzthiazide
albuterol atropine brimonidine
aluminum nicotinate(?) azatadine brompheniramine
amitriptyline belladonna busulfan
antazoline bendroflumethiazide butaperazine
carbinoxamine hydroflumethiazide pimozide
carphenazine imipramine pindolol
chlorisondamine indapamide piperacetazine
chlorothiazide isotretinoin polythiazide
chlorpheniramine lithium carbonate practolol
chlorpromazine marihuana prochlorperazine
chlorthalidone mesoridazine promazine
cimetidine(?) methdilazine promethazine
clemastine fumarate methotrexate propiomazine
clonidine methotrimeprazine propranolol
cyclothiazide methoxsalen protriptyline
cyproheptadine methscopolamine pyrilamine
desipramine methyclothiazide quinethazone
dexbrompheniramine methyldopa scopolamine
dexchlorpheniramine methylthiouracil tartrate
diethazine metolazone tetrahydrocannabinol
dimethindene metoprolol thiethylperazine
diphenhydramine morphine thiopropazate
diphenylpyraline nadolol thioproperazine
disopyramide niacin(?) thioridazine
doxylamine niacinamide(?) timolol
dronabinol nicotinyl alcohol(?) tolterodine
ether nitrous oxide trichlormethiazide
ethopropazine nortriptyline trichloroethylene
etretinate opium trifluoperazine
fluphenazine oxprenolol triflupromazine
hashish perazine trimeprazine
hexamethonium periciazine trioxsalen
homatropine perphenazine tripelennamine
hydrochlorothiazide pheniramine triprolidine
(7) Felty syndrome (uveitis-rheumatoid arthritis syndrome)

(8) Gougerot-Sjögren syndrome (oligophrenia-ichthyosis-spastic diplegia
syndrome)
(9) Heerfordt syndrome (uveoparotitis)
(10) Lubarsch-Pick syndrome (primary amyloidosis)
(11) Mikulicz syndrome-acryoadenitis and parotitis
a. Hodgkin disease
b. Leukemia
c. Lymphoma
d. Lymphosarcoma
e. Mumps
f. Sarcoidosis syndrome (Schaumann syndrome)
g. Syphilis
h. Tuberculosis
i. Waldenstrom macroglobulinemia
(12) Pancreatitis
(13) Pheochromocytoma, medullary thyroid carcinoma, and multiple
mucosal neuromas
(14) Polyarteritis nodosa (Kussmaul disease)
(15) Relapsing polychondritis
(16) Rheumatoid arthritis (adult)
(17) Scleroderma (progressive systemic sclerosis)
Geier SA. Sicca syndrome in patients infected with the human immunodeficiency virus.
Ophthalmology 1995; 102: 1319-1324.
1992.
DACRYOCYSTITIS (INFECTION OF THE LACRIMAL SAC)
1. Acute dacryocystitis
*A. Beta-hemolytic streptococcus
B. Corynebacterium diphtheriae
C. Dacryolith
D. Erysipelothrix insidiosa
E. Friedlander bacillus
F. Fusobacterium (canaliculitis and dacryocystitis)
G. Granulomatous "pseudotumor"
*H. Haemophilus aegyptius (Koch-Weeks bacillus)
I. Infectious mononucleosis
J. Influenza
K. Lymphocytic neoplasia
L. Neisseria catarrhalis
M. Pasteurella multocida
*N. Pneumococcus
*O. Pseudomonas aeruginosa
P. Rhinosporidiosis
Q. Rubeola (measles)
R. Serratia marcescens - gram-negative coccobacillus
*S. Staphylococcus
T. Streptococcus
U. Tularemia
V. Variola
2. Chronic dacryocystitis
A. Associated with osteopoikilosis
B. Actinomyces israelii
C. Aspergillus
D. Bacillus fusiformis
E. Candida albicans
F. Escherichia coli
G. Lymphoma of the lacrimal sac
H. Mycobacterium fortuitum and Mycobacterium chelonei
I. Nocardia asteroides
J. Francisella tularensis
K. Mycobacterium leprae
L. Proteus vulgaris
M. Sporotrichosis
N. Syphilis (acquired lues)
O. Systemic sarcoidosis
P. Thermal bums
Q. Trachoma
R. Treponema vincentii
S. Tuberculosis (Mycobacterium tuberculosis)
T. Wegener granulomatosis
Artenstein AW, et al. Chronic dacryocystitis caused by Mycobacterium fortuitum.
Ophthalmology 1993; 100: 666-668.
Gunal I, et al. Dacryocystitis associated with osteopoikilosis. Clin Genet 1993; 44:211-
213.
Haynes BF, et al. The ocular manifestations of Wegener's granulomatosis: fifteen year's
experience and review of the literature. Am J Med 1977; 63:131.
Karesh JW, et al. Dacryocystitis associated with malignant lymphoma of the lacrimal sac.
4
Extraocular Muscles
CONTENTS
Pseudoesotropia 128
Esophoria and esotropia 128
Pseudoexotropia 129
Exophoria and exotropia 130
Pseudohypertropia 130
Hyperphoria and hypertropia 130
Brown superior oblique tendon sheath syndrome 132
Duane syndrome 133
Oculomotor apraxia 133
Monocular limitation of elevation of adducted eye with forced duction test 134
Cyclic, recurrent, repetitive, episodic disorders of extraocular muscles 134
Syndromes and diseases associated with strabismus 135
Horizontal gaze palsy 139
Oscillations of eyes 140
Cogwheel eye movements 141
Pendular nystagmus 142
Horizontal jerk nystagmus 142
Vertical nystagmus 143
Rotary nystagmus 144
See-saw nystagmus 144
Retraction nystagmus 145
Monocular nystagmus 145
Periodic alternating nystagmus 145
Positional nystagmus 146
Optokinetic nystagmus 148
Syndromes and diseases associated with nystagmus 148
Oculogyric crisis 152
Ocular bobbing 153
Paralysis of third nerve 153
Childhood causes of third nerve (oculomotor) palsy 157
Paralysis of fourth nerve 158
Childhood causes of fourth nerve (trochlear) palsy 160
Pseudoabducens palsy 160
Paralysis of sixth nerve 161
Paralysis of sixth nerve 162
Paralysis of third, fourth, and sixth cranial nerves 164
Childhood causes of sixth nerve (abducans) palsy 167
Childhood causes of third, fourth, and sixth nerve palsy 167
Acute ophthalmoplegia 168
Chronic ophthalmoplegia 171
Bilateral complete ophthalmoplegia 171
External ophthalmoplegia 172
Internuclear ophthalmoplegia 173
Painful ophthalmoplegia 173
Transient ophthalmoplegia 176
Painful ocular movements 176
Poor convergence 177
Spasm of convergence 178
Divergence paralysis 178
Oculocardiac reflex 179
Retraction of the globe 179
Forced duction test 180
Double elevator palsy 181
Ocular neuromyotonia 181
Extraocular muscle enlargement 181
PSEUDOESOTROPIA (OCULAR APPEARANCE OF ESOTROPIA WHEN NO

MANIFEST DEVIATION OF VISUAL AXIS IS PRESENT)
* 1. Abnormal shape of skull or abnormal thickness of skin surrounding the orbits

2. Enophthalmos
3. Entropion (p. 79)
4. Hypotelorism with narrow interpupillary distance
5. Lateral displacement of the concavity of the upper eyelid margin from the center of the
pupil
*6. Negative-angle kappa-pupillary light reflex displaced temporally (see Decentered
Pupillary Light Reflex, p. 361)
*7. Prominent epicanthal fold
8. Telecanthus-the orbits are normally placed, but the medial canthi are far apart
secondary to lateral displacement of the soft tissues
Shaterian ET, Weismann IL. An unusual case of pseudostrabismus. Am Orthop J 1973;

23:68-70.
Urist MJ. Pseudostrabismus caused by abnormal configuration of the upper eyelid

margins. Am J Ophthalmol 1973; 75:455-456.
ESOPHORIA AND ESOTROPIA (VISUAL AXIS DEVIATED INWARD; MAY

BE LATENT OR MANIFEST)
1. Comitant (nonparalytic)-angle of deviation is constant in all directions of gaze
A. Accommodative-hyperopic refractive error
B. Nonaccommodative-refractive error not cause of deviation
(1) Anomalous insertion of horizontally acting muscles
(2) Abnormal check ligaments
(3) Faulty innervational development
(4) Autosomal recessive trait
(5) Idiopathic
(6) Tumor of the brain
a. Cerebellar astrocytoma
b. Pontine glioma
2. Noncomitant-the angle of deviation varies in different directions of gaze
A. Abducens palsy (p. 161)
B. Accommodative spasm
C. Blowout fracture
D. Divergence paralysis
E. Drug use (marihuana)
F. Duane syndrome
G. Myasthenia gravis
H. Thyroid myopathy
3. "V" pattern esotropia-deviation greater in downward gaze
A. Underaction-superior oblique muscles
B. Overaction-inferior oblique muscles
4. "A" pattern esotropia
A. Underaction-inferior oblique muscles
5. Monocular esotropia-one eye may be used to the exclusion of the other; amblyopia is
usual in the deviating eye
6. Esotropia-near/distance disparity
A. High accommodation convergence-accommodation (AC/ A) ratio-greater
convergence for near than for distance, causing greater esodeviation for near than
for distance
B. Convergence excess-greater esodeviation for near than for distance
C. Divergence insufficiency-greater esodeviation for distance than for near
Helveston EM. The origins of congenital esotropia. J Pediatr Ophthalmol Strabismus

1993; 30:215-232.
von Noorden GK. Binocular vision and ocular motility: theory and management of
strabismus. St. Louis: CV Mosby, 1995.
Wright KW. Pediatric ophthalmology and strabismus. St. Louis: CV Mosby, 1995.
Williams AS, Hoyt CS. Acute comitant esotropia in children with brain tumors. Arch
PSEUDOEXOTROPIA (OCULAR APPEARANCE OF EXOTROPIA WHEN NO

MANIFEST DEVIATION OF VISUAL AXIS IS PRESENT)
1. Displaced macula (heterotopia of the macula; see p. 451)

2. Heterochromia when the lighter-colored eye appears to diverge (see p. 368-372)
3. Hypertelorism with wide interpupillary distance
4. Exophthalmos
5. Positive-angle kappa-pupillary light reflex displaced nasally (see decentered papillary
light reflex, p. 361)
6. Narrow lateral canthus
7. Wide palpebral fissure
Beyer-Machule C, von Noorden GK. Atlas of ophthalmic surgery. Vol 1: Lids, orbits,
extraocular muscles. New York: Thieme Medical, 1984.
Shaterian ET, Weissman IL. An unusual case of pseudostrabismus. Am Orthopt J 1973;

23:68-70.
EXOPHORIA AND EXOTROPIA (VISUAL AXIS DEVIATED OUTWARD; MAY

BE LATENT OR MANIFEST)
1. Comitant
A. Refractive-myopic refractive error cause of deviation (low AC/A ratio)
B. Nonrefractive-refractive error not cause of deviation
C. Anomalous insertion of horizontally acting muscles
D. Abnormal check ligaments
E. Faulty innervational development
F. Autosomal-dominant trait
G. Idiopathic
2. Noncomitant
A. Convergence insufficiency
B. Divergence excess
C. Duane type II, III
D. Internuclear ophthalmoplegia
E. Myasthenia gravis
F. Third nerve palsy
G. Thyroid myopathy
3. Pattern exotropia
A. V exotropia-deviation greater in upward than in downward gaze
(1) Underaction superior oblique
(2) Overaction inferior oblique
B. Exotropia-deviation greater in downward than in upward gaze
(1) Underaction inferior oblique muscle
(2) Overaction superior oblique muscle
von Noorden GK. Binocular vision and ocular motility: theory and management of
strabismus. St. Louis: CV Mosby.
PSEUDOHYPERTROPIA
1. Facial asymmetry with one eye placed higher than the other
2. Unilateral coloboma of lid
3. Unilateral ptosis
Shaterian ET, Weissman IL. An unusual case of pseudostrabismus. Am Orthop J 1973;

23:68-70.
Wright DW. Pediatric ophthalmology and strabismus. St. Louis: CV Mosby, 1995.
HYPERPHORIA AND HYPERTROPIA (VISUAL AXIS DEVIATED UPWARD;

MAYBE MANIFEST OR LATENT)
1. Nonparalytic hypertropia
A. Abnormal insertion of muscles
B. Abnormal fascial attachments
C. Complications of systemic diseases, such as myasthenia gravis, thyrotoxicosis,
and orbital tumors
2. Paralytic hypertropia-isolated cyclovertical muscle palsy
A. Brainstem disease
B. Fourth nerve palsy
C. Multiple sclerosis
D. Skew deviation
E. Third nerve palsy
3. Double hyperphoria (alternating circumduction)-fuses, but cover test shows alternating
hyperphoria
4. Apparent paralysis of elevation of one eye
A. Local neuromuscular and orbital causes
(1) Dysthyroid ophthalmoplegia (noncongestive and congestive form)
(2) Myasthenia gravis (Erb-Goldflam syndrome)
(3) Orbital floor fracture
(4) Progressive supra nuclear ophthalmoplegia
(5) Oculomotor nerve paresis superior division
(6) Unilateral double-elevator palsy, congenital dysfunction of superior
rectus and inferior oblique muscles
(7) Myositis
a. "Collagen diseases"
b. Infectious myositis
c. Trichinosis
(8) Orbital tumors
a. Dermoid cyst
b. Hemangioma
c. Lymphoma
d. Meningioma
e. Optic nerve glioma
f. Previous strabismus surgery
g. Rhabdomyosarcoma
(9) Systemic amyloidosis with ocular muscle infiltration
(10) Vertical retraction syndrome (Parinaud syndrome)
(11) Superior oblique tendon sheath syndrome (Brown syndrome)
B. Skew deviation due to a central nervous system lesion-one eye is above the
other; may be the same for all directions of gaze or vary in different directions of
gaze
(1) Unilateral labyrinthine disease
(2) Cerebellar tumors, such as astrocytomas and medulloblastomas
(3) Acoustic neuromas
(4) Vascular accidents of pons and cerebellum, such as thrombosis of
cerebellar and pontine arteries
(5) Unilateral internuclear ophthalmoplegia and less frequently bilateral
internuclear ophthalmoplegia
(6) Compressive lesions, such as platybasia and Arnold-Chiari
malformation
(7) Brain-stem arteriovenous malformations
(8) Aberrant regeneration of third nerve
C. Central nervous system lesions
(1) Arteriosclerosis, thrombosis, arteritis (syphilitic), or embolus of fine
vessels to midbrain
5. Apparent paralysis of elevation of both eyes
A. Physiologic in older persons
B. Parinaud syndrome (divergence paralysis)
C. Chronic progressive external ophthalmology (CPEO)
F. Midbrain lesion
(1) Upgaze center
(2) Bilateral third nerve palsy
(3) Dorsal midbrain syndrome
G. Congenital fibrous syndrome
H. Thyroid myopathy
I. Metastatic tumor (breast cancer)
6. Paralysis of downward gaze
A. Reverse Parinaud syndrome
B. Associated with choreoathetotic syndromes
C. Parkinsonian syndromes
D. Myasthenia gravis
E. Miscellaneous
Flaherty MP, et al. Congenital fibrosis of the extraocular muscles associated with cortical
dysplasia and maldevelopment of the basal ganglia. Ophthalmology 2001; 108:1313-
1322.
Keane JR. Ocular skew deviation. Arch Neurol 1975; 32:185.

Metz HS. Double elevator palsy. J Ped Ophthal Strab 1981; 18:31-36.
Kushner BJ. Errors in the three-step test in the diagnosis of vertical strabismus.
Ophthalmol 1989; 96:127-132.
BROWN SUPERIOR OBLIQUE TENDON SHEATH SYNDROME

(LIMITATION OF ELEVATION IN ADDUCTION THAT RESEMBLES AN
UNDERACTION OF INFERIOR OBLIQUE MUSCLE)
1. Congenital onset
A. Congenital Brown syndrome
B. Inelastic muscle-tendon complex
2. Anomalies of Superior Oblique Tendon fibers
3. Congenital pseudo-Brown Syndrome-anomalous inferior orbital adhesions
4. Posterior orbital bonds
5. Acquired onset
A. Acquired Brown syndrome
B. Peritrochlear scarring and adhesions
C. Chronic sinusitis
6. Trauma-superior temporal orbit
7. Blepharoplasty and fat removal
8. Lichen sclerosis at astrophicus and morpheae
9. Tendon-trochlear inflammation and edema
10. Idiopathic inflammatory (pain and click)
11. Trochlearitis with superior oblique myositis
12. Acute sinusitis
13. Adult rheumatoid arthritis
14. Juvenile rheumatoid arthritis
15. Systemic lupus erythematosus
16. Possibly distant trauma (CPR [cardiopulmonary resusitation] and long-bone fractures)
17. Possibly postpartum hormonal change
18. Superior nasal orbital mass
19. Glaucoma implant
20. Neoplasm
21. Tight or inelastic superior oblique muscles
22. Thyroid disease (inelastic muscles)
23. Peribulbar anesthesia (inelastic tendon)
24. Hurler Scheie syndrome (inelastic tendon)
25. Superior oblique tuck (short tendon)
26. Idiopathic
27. Acquired pseudo-Brown syndrome
28. Orbital fracture
29. Retinal band around inferior oblique muscle
30. Inferior temporal adhesion
31. Following double plate Molteno implantation
Dobler-Dixon AA, et al. Prospective evaluation of extraocular motility following double-
plate molteno implantation. Arch Ophthalmol 1999; 117: 1155-1160.
Kaban JT, et al. Natural history of presumed congenital Brown's syndrome. Arch
Wang FM, et al. Brown's syndrome in children with juvenile rheumatoid arthritis.
Wilson ME, et al. Brown's syndrome. Surv Ophthal 1989; 34:153.
DUANE SYNDROME
Congenital ocular motility disorder is characterized by limited abduction or
limited adduction. The palpebral fissure narrows on attempted adduction.
1. Type 1 characteristics
a. A or V phenomena
b. Defective abduction
c. Palpebral fissure narrowing on adduction
d. Retraction of the globe
e. Updrift or downdrift of the affected eye on adduction or attempted abduction
a. Abduction appears to be normal or only slightly limited
b. Distinct narrowing of the palpebral fissure and retraction of the globe on
attempted adduction.
c. Limitation or complete palsy of adduction with exotropia of the paretic eye.
a. Limitation or absence of both abduction and adduction of the affected eye
b. Globe retraction and narrowing of the palpebral fissure on attempted adduction
Duane A. Congenital deficiency of abduction associated with impairment of adduction,

retraction movements, contraction of the palpebral fissure, and oblique movements of the
eye. Arch Ophthalmol 1996; 114: 1255-1256.
Duane TD, et al. Pseudo-Duane's retraction syndrome. Trans Am Ophthalmol Soc 1977;
74:122-132.
OCULOMOTOR APRAXIA
Oculomotor apraxia is defined as defective or absent horizontal voluntary eye
movements and includes head thrusting to look at objects to the side.
1. Ataxia-telangiectasia syndrome
2. Brain tumor
A. Astrocytoma
B. Lipoma
3. Isolated
4. Male predominance
6. Oral-facial-digital syndrome type II
7. Post cardiac surgery
Moschner C, et al. Comparison of oculomotor findings in the progressive ataxia

syndromes. Brain 1995; 117:15-25.
Zackon DH, Noel L. Ocular motor apraxia following cardiac surgery. Can J Ophthal
1991; 26:102.
Zaret C, et al. Congenital ocular motor apraxia and brainstem tumor. Arch Ophthalmol
1980; 98:328-330.
MONOCULAR LIMITATION OF ELEVATION OF ADDUCTED EYE WITH

FORCED DUCTION TEST [IN ELEVATION AND ADDUCTION STRABISMUS
WITH RESTRICTED MOTILITY]
1. Acquired
A. Thyroid myopathy
*B. Excessive recession or resection of muscle
C. Orbital fracture
D. Retinal detachment operation
E. Strabismus surgery complicated by adhesions
F. CPEO
2. Congenital
A. Congenital fibrous syndrome
B. Neurogenic paralysis with secondary contracture of antagonist muscle
C. Duane retraction syndrome
D. Brown superior oblique tendon sheath syndrome
E. Strabismus fixus
Harley RD, et al. Congenital fibrosis of the extraocular muscles. Trans Am Ophthal Soc
1978; 76:197.
CYCLIC, RECURRENT, REPETITIVE, EPISODIC DISORDERS OF

EXTRAOCULAR MUSCLES
1. Cyclic strabismus
A. Associated with frontoorbital fibrous dysplasia
B. Associated with Graves disease
C. Associated with optic atrophy
D. Cyclic superior oblique palsy
E. Cyclic third nerve palsy
F. Esotropia, vertical
(1) Comitant
(2) Noncomitant
2. Cyclic vertical deviation
3. Diabetic nerve palsies
4. Myasthenia gravis
5. Oculogyric crisis (see p. 152)
6. Periodic alternating gaze deviation
7. Periodic alternating nystagmus
8. Periodic vertical nystagmus
A. Associated with potassium abnormality
B. Familial
9. Petit mal epilepsy
A. Exotropia
B. Upward deviation
10. Ping-pong gaze
11. Recurrent sixth nerve paralysis in children (see p. 169)
12. Spasmus nutans
13. Twitch of lids (orbicularis)
Hamed L. Cyclic periodic disorders in diagnostic problems in clinical ophthalmology.

Margo CL, ed. Philadelphia: WB Saunders, 1994.
Hoyt WF, Keane JR. Superior oblique myokymia. Arch Ophthalmol 1970; 84:461-467.
Windsor CE, Berg EF. Circadian heterotropia. Am J Ophthalmol 1969; 67:565-571.
SYNDROMES AND DISEASES ASSOCIATED WITH STRABISMUS
1. Esotropia syndrome
2. Exotropia syndrome
3. Aarskog syndrome (facial-digital-genital syndrome)
4. Aberfeld syndrome (congenital blepharophimosis associated with generalized
myopathy)
5. Achondroplasia
6. Addison pernicious anemia
7. African eyeworm disease
8. Albinism
9. Albright hereditary osteodystrophy (pseudohypoparathyroidism)
10. Amyloidosis
12. Arnold-Chiari syndrome (platybasia syndrome)
13. Arylsulfatase A deficiency syndrome
14. Aspergillosis
15. Axenfeld-Schurenberg syndrome (cyclic oculomotor paralysis)
16. Bacterial endocarditis
17. Bang disease (brucellosis)
18. Behçet syndrome (oculobuccogenital syndrome)
19. Benedikt syndrome (tegmental syndrome)
20. Best disease (vitelliform dystrophy)
21. Bielschowsky-Lutz-Cogan syndrome (internuclear ophthalmoplegia)
22. Bing-Neel syndrome (associated with macroglobulinemia and central nervous system
symptoms)
23. Bloch-Sulzberger disease (incontinentia pigmenti)
24. Blocked nystagmus syndrome (nystagmus blockage syndrome)
26. Bonnevie-Ullrich syndrome (pterygolymphangiectasia)
27. Botulism
28. Brown-Marie syndrome (hereditary ataxia syndrome)
29. Canine tooth syndrome (class VII superior oblique palsy)
30. Cerebral palsy
31. Chediak-Higashi syndrome (anomalous leukocytic inclusions with constitutional
stigmata)
32. Chromosome partial deletion (short-arm) syndrome (Wolf syndrome)
33. Chromosome 13q partial deletion (long-arm) syndrome (thirteen Q syndrome)
34. Chromosome partial deletion (long-arm) syndrome (DeGrouchy syndrome)
35. Chromosome partial (short-arm) partial deletion syndrome
36. Congenital syphilis
37. Convergence insufficiency syndrome
38. Craniocarpotarsal dysplasia (Freeman-Sheldon syndrome; whistling face syndrome)
39. Craniostenosis
41. Crohn disease (granulomatous ileocolitis)
43. Cushing syndrome (II) (cerebellopontine angle syndrome)
44. Cysticercosis
45. Cytomegalic inclusion disease, congenital
47. De Lange syndrome (congenital muscular hypertrophy-cerebral syndrome)
48. Dengue fever
49. Devic syndrome (ophthalmoencephalomyelopathy)
50. Diabetes mellitus
51. Diphtheria
52. Diamond-Blackfan syndrome
53. Down disease (mongolism, trisomy 21)
alcohol chloroform iothalamate meglumine
baclofen cholecalciferol and sodium
calcitriol ergocalciferol iothalamic acid
chloramphenicol(?) insulin isocarboxazid
measles and rubella mumps virus vaccine rubella virus vaccine
virus vaccine (live) (live) (live)
measles virus vaccine nialamide tranylcypromine
(live) pemoline tripelennamine
measles, mumps, and pentylenetetrazol vitamin A
rubella virus vaccine phenelzine vitamin D2
(live) rubella and mumps virus vitamin D3
metoclopramide vaccine (live)
metrizamide
54. Drummond syndrome (idiopathic hypercalcemia)

56. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)
57. Ehlers-Danlos disease (fibrodysplasia elastica generalisata)
59. Ellis-van Creveld syndrome (chondroectodermal dysplasia)
61. Engelmann syndrome (osteopathia hyperostotica scleroticans multiplex infantalis)
63. Erb-Goldflam disease
65. Fibrosarcoma
66. François dyscephalic syndrome
67. Gaucher syndrome (glucocerebroside storage disease)
68. Gangliosidosis
A. Infantile (GM1)
B. Juvenile (GM2)
69. Goltz syndrome (focal dermal hypoplasia syndrome)
70. Gorlin-Goltz syndrome (multiple basal cell nevi syndrome)
71. Greig syndrome (ocular hypertelorism syndrome)
72. Grönblad-Strandberg syndrome (systemic elastodystrophy)
73. Hemangiomas
74. Hemifacial hyperplasia with strabismus (Bencze syndrome-autosomal dominant)
75. Hemifacial microsomia (otomandibular dysostosis)
76. Homocystinuria
77. Hurler disease (mucopolysaccharidoses type I)
78. Hutchinson syndrome (adrenal cortex neuroblastoma with orbital metastasis)
79. Hydrocephalus, congenital
80. Hydrophobia (rabies)
81. Hyperthyroidism
82. Hypocalcemia
83. Hypomelanosis of Ito syndrome (incontinentia pigmenti achromians)
85. Hysteria
87. Influenza
89. Johnson syndrome (adherence syndrome)
90. Klippel-Feil syndrome (congenital brevicollis)
91. Koerber-Salus-Elschnig syndrome (nystagmus retractorius syndrome)
92. Kohn-Romano syndrome (telecanthus, ptosis, epicanthus inversus, blepharophimosis)
94. Kugelberg-Welander syndrome (progressive muscle atrophy)
96. Larsen syndrome (hypertelorism, microtia, and facial clefting)
99. Leukemia
101. Lowe syndrome (ocu1ocerebrorenal syndrome)
102. Lymphangioma
103. Lymphedema
104. Lymphoid hyperplasia (Burkitt lymphoma)
105. Malaria
106. Malignant hyperpyrexia syndrome
108. Maple syrup urine disease
109. Marcus Gunn syndrome (jaw-winking syndrome)
110. Marfan syndrome (arachnodactyly-dystrophia mesodermalis congenita)
111. Measles
112. Melnick - Needles syndrome (osteodysplasty)
113. Mieten syndrome (corneal opacity, nystagmus, flexion contracture, growth failure)
114. Millard-Gubler syndrome (abducens-facial hemiplegia alternans)
115. Möbius syndrome (congenital paralysis of sixth or seventh nerves)
116. Monofixation syndrome (blind-spot syndrome)
117. Morning glory syndrome (hereditary central glial anomaly of the optic disk)
118. Mucocele
119. Mucormycosis
120. Mulibrey nanism syndrome (Perheentupa syndrome)
121. Multiple lentigines syndrome (leopard syndrome)
122. Multiple sclerosis
123. Mumps
125. Naegeli syndrome (melanophoric nevus syndrome)
126. Nematode ophthalmia syndrome (toxocariasis)
127. Neonatal hemolytic disease of hyperbilirubinemia
128. Neuroblastoma
129. Nevus sebaceous of Jadassohn
130. Nevoid basal cell carcinoma syndrome
131. Nielsen syndrome (exhaustive psychosis syndrome)
133. Noone-Milroy-Meige disease (congenital trophedema)
134. Nothnagel syndrome (ophthalmoplegia cerebellar ataxia syndrome)
135. Nystagmus compensation syndrome
136. Obesity-cerebrolocular-skeletal anomalies syndrome
137. Ocular vaccinia
138. Oculocerebellar tegmental syndrome
139. Oculo-oto-ororenoerythropoietic syndrome
140. Ophthalmoplegic retinal degeneration syndrome
141. Orbital floor syndrome (Dejean syndrome)
144. Papillon-Léage and Psaume syndrome (orodigital facial syndrome)
145. Parkinson syndrome
146. Parry-Romberg disease (progressive facial hemiatrophy)
148. Pertussis (whooping cough)
149. Pierre Robin syndrome (micrognathia-glossoptosis syndrome)
150. Polymyalgia rheumatica
151. Postvaccinial ocular syndrome
152. Pseudoophthalmoplegia syndrome (Roth-Bielschowsky syndrome)
153. Prader-Willi syndrome (hypotonia-obesity syndrome)
154. Pseudohypoparathyroidism (Seabright-Bantam syndrome)
155. Reiter syndrome (conjunctivourethrosynovial syndrome)
156. Relapsing fever
157. Retinoblastoma
158. Ring chromosome 18
160. Ring dermoid syndrome
161. Rocky Mountain spotted fever
162. Rubella, congenital
163. Rubinstein- Taybi syndrome (broad-thumb syndrome)
164. Sabin-Feldman syndrome
165. Sandifer syndrome (hiatus hernia-torticollis syndrome)
166. Schilder syndrome (encephalitis periaxialis diffusa)
167. Seckel bird-headed dwarfism
168. Skew deviation syndrome
169. Smallpox
171. Spongy degeneration of the white matter
172. Streptococcus
173. Superior oblique tendon sheath syndrome (Brown syndrome)
174. Supravalvular aortic stenosis syndrome (infantile hypercalcemia with mental
retardation)
175. Tay-Sachs syndrome (familial amaurotic idiocy)
176. Temporal arteritis syndrome (cranial arteritis syndrome)
177. Terson syndrome (subarachnoid hemorrhage syndrome)
178. Thomsen syndrome (congenital myotonia syndrome)
179. Trichinosis
Hayashi N, et al. Ocular histopathologic study of a patient with the T 8993-G point
mutation in Leigh's syndrome. Ophthalmology 2000; 107:1397-1402.
HORIZONTAL GAZE PALSY

Horizontal gaze palsy comprises an inability to look horizontally in a given
direction; analysis includes optically induced movement, voluntary or command
movement, pursuit movement, or vestibular movement.
1. Horizontal palsy of voluntary and command movement-frontal lobe gaze center

(second frontal gyrus, Brodmann area 8) or in the corresponding internal capsule gaze
palsy of side opposite lesion; it may be associated with facial palsy as well as hemiparesis
or hemiplegia toward the side of the gaze palsy, caloric ocular movement intact, and
doll's head intact.
2. Horizontal palsy of command and pursuit movements, optically induced movements,
and vestibular movements-pons and posterior longitudinal bundle; the gaze palsy is
toward the side of the lesion, facial palsy often present, caloric and doll's head responses
are absent.
Huber A. Eye symptoms in brain tumors, 2nd ed. St. Louis: CV Mosby, 1971:44-47.
OSCILLATIONS OF EYES (INVOLUNTARY, RAPID, TO-AND-FRO

MOVEMENT OF EYES HAVING NO RHYTHM OR REGULARITY)
1. Ocular dysmetria- "overshooting" of the eyes with attempted fixation; horizontal

ocular dysmetria is associated with lesions of the cerebellum or its pathways as in
Friedreich ataxia, Huntington chorea, spinocerebellar degeneration, internuclear
ophthalmoplegia, manic depression, alcoholism, schizophrenia, severe diffuse brain
damage, cerebellopontine angle tumors, hereditary ectodermal dysplasia with
olivopontocerebellar degeneration, Fabry disease (glycosphingolipid lipidosis),
vestibulocerebellar ataxia, and toluene abuse
2. Ocular flutter-flutter-like oscillations that are intermittent, rapid, to-and-fro motions, or
motions of equal amplitude, interrupt maintained fixation; horizontal ocular flutter is
associated with lesions of the cerebellum or its pathways as in limb ataxia, multiple
sclerosis, poliomyelitis, neoplasms, or vascular accident
3. Opsoclonus-irregular, hyperkinetic, multidirectional, spontaneous eye movement that
persists in sleep
A. Infections
(1) Coxsackie B3
(2) Encephalitis-mild, severe, viral or post infections (including St. Louis
encephalitis)
(3) Haemophilus influenzae
(4) Meningitis
(5) Paratyphi A
(6) Psittacosis
(7) Salmonella
B. Tumors
(1) Breast malignancy
(2) Bronchogenic carcinoma
(3) Glioblastoma
(4) Neuroblastoma
(5) Thyroid carcinoma
(6) Uterine carcinoma
C. Toxins and drugs
(1) Amitriptyline
(2) Chlordecone
(3) Lithium-haloperidol (Haldol)
(4) Thallium
(5) Toluene abuse
D. Other
(1) Acute cerebellar ataxia
(2) Friedreich ataxia
(3) Multiple sclerosis (disseminated sclerosis) (rare)
(4) Nonketotic coma
(5) Sign of "myoclonic encephalopathy of infancy"
(6) Vascular accidents
(7) Vertebrobasilar insufficiency
4. Lightning eye movements (ocular myoclonus)-rapid to-and-fro movements of small
conjugate saccades; probably because of bilateral abnormality of a pontine paramedical
zone and pretectal lesions, such as vascular, inflammatory, neoplastic, demyelinating, or
trauma of tegmentum as thyroid, lung or uterus carcinoma, neuroblastoma, Menzel
hereditary ataxia, pontine myelinolysis, coxsackie B infection, cherry-red spot myoclonus
syndrome, Ramsay-Hunt syndrome, and L-tryptophan
Dropcho E, Payne R. Paraneoplastic opsoclonus myoclonus: association with medullary

thyroid carcinoma and review of the literature. Arch Neurol 1986:43:410-415.
Farris BK, et al. Neuro-ophthalmologic findings in vestibulocerebellar ataxia. Arch

Neurol 1986; 43:1050-1053.
Lazar RB, et al. Multifocal central nervous system damage caused by toluene abuse.
Neurology 1983; 33: 1337-1340.
Matsumura K, et al. Syndrome of opsoclonus-myoclonus in hyperosmolar nonketotic

coma. Ann Neurol 1985; 18: 623-624.
COGWHEEL EYE MOVEMENTS (JERKY INACCURATE PURSUIT

MOVEMENTS)
1. Basal ganglia disease
A. Anoxia
B. Carbon disulfide poisoning
C. Carbon monoxide poisoning
D. Drugs, including:
acetophenazine etidocaine prazepam
alcohol fluphenazine prilocaine
allobarbital flurazepam primidone
alprazolam halazepam probarbital
alseroxylon heptabarbital procaine
amitriptyline hexethal prochlorperazine
amobarbital hexobarbital promazine
aprobarbital imipramine promethazine
barbital lidocaine propiomazine
bromide lithium carbonate propoxycaine
bupivacaine lorazepam protriptyline
butabarbital mephobarbital rauwolfia serpentina
butalbital mepivacaine rescinnamine
butallylonal mesoridazine reserpine
butaperazine metharbital secobarbital
butethal methdilazine syrosingopine
carphenazine methitural talbutal
chloral hydrate methohexital temazepam
chlordiazepoxide methotrimeprazine thiamylal
chloroprocaine midazolam thiethylperazine
chlorpromazine nitrazepam thiopental
clonazepam nortriptyline thiopropazate
clorazepate oxazepam thioproperazine
cyclobarbital pentobarbital thioridazine
cyclopentobarbital perazine triazolam
deserpidine periciazine trifluoperazine
desipramine perphenazine triflupromazine
diazepam phencyclidine trimeprazine
diethazine phenobarbital vinbarbital
ethopropazine piperacetazine
E. Exposure to manganese
F. Idiopathic
G. Parkinsonism (shaking palsy)
H. Trauma
2. Cerebellar tumors
A. Astrocytomas
B. Hemangioblastomas
C. Medulloblastomas
3. With homonymous hemianopia, indicates parietal or occipital lobe involvement
Wilkins, 1985.
PENDULAR NYSTAGMUS
Pendular nystagmus comprises oscillations that are approximately equal in rate in
two directions; they may be horizontal or vertical.
1. Albinism in which the macula does not develop

2. Aniridia (see p. 364-365)
3. Bilateral chorioretinal lesions involving the macula in early infancy (congenital
toxoplasmosis)
4. Brainstem or cerebellar dysfunction
*5. Congenital-cause unknown, may be inherited as autosomal dominant recessive or X-
linked recessive trait; not infrequently associated with astigmatism and convergent
strabismus
*6. Congenital cataracts
7. Congenital glaucoma
8. Corneal scars
9. Demyelinating disease
10. High myopia of early life
11. Internuclear ophthalmoplegia
13. Leber congenital amaurosis
14. Monocular or binocular visual deprivation
15. Optic nerve hypoplasia, coloboma
*16. Total color blindness (monochromatism)
17. Work in poor illuminations (e.g., mining) (rare)
Cogan DG. Neurology of the ocular muscles, 4th ed. Springfield, IL: Charles C Thomas,
1978.
Wilkins, 1985.
HORIZONTAL JERK NYSTAGMUS (HORIZONTAL OSCILLATORY

MOVEMENT OF EYES WITH A FAST AND SLOW PHASE)
1. Albinism
2. Amblyopia (manifest latent nystagmus)
3. Cerebellar disease, acute or chronic; fast component to side of lesion
stigmata)
5. Congenital achromatopsia
6. Congenital cataracts
7. Congenital stationary night-blindness
8. Congenital X-linked, dominant, recessive
10. Lesions of labyrinth (e.g., Meniere syndrome) or when one labyrinth has been
removed
11. Neoplastic angioendotheliomatosis
12. Optic nerve hypoplasia, coloboma
13. Vestibular nuclei involvement as in persons with multiple sclerosis
Barton JJ, Sharpe JA. Oscillopsia and horizontal nystagmus with accelerating slow
phases following lumbar puncture in the Arnold-Chiari malformation. Ann Neurol 1993;
33:418-421.
Cogan DG. Neurology of the ocular muscles, 4th ed. Springfield, IL: Charles C Thomas,
1978.
Elner VM, et al. Neoplastic angioendotheliomatosis. Ophthalmology 1986; 93:1237-

1245.
Spaeth GL. Ocular manifestations of the lipidoses. In: Tasman WC, ed. Retinal disease in
children. New York: Harper & Row, 1971:187-188.
VERTICAL NYSTAGMUS (SPONTANEOUS VERTICAL OSCILLATIONS OF

EYES)
1. Upbeat nystagmus-nystagmus in which the fast component is upward and usually most
marked when the gaze is directed upward; usually due to a lesion in the posterior fossa
A. Brainstem lesion, such as that of the vestibular nuclei
B. Cerebellar disease-acute or chronic, especially in the vermis
C. Cerebellar degeneration
D. Drugs-barbiturates and Dilantin (phenytoin)
E. Encephalitis
F. Labyrinth disease-rare; has no lateralizing value
G. Multiple sclerosis
H. Idiopathic
2. Downbeat nystagmus-nystagmus in which the fast component is downward and
usually most marked when the gaze is directed downward; probably due to a lesion in the
lower end of the brain stem or cerebellum
A. Alcoholic cerebellar disease
B. Aneurysm of the supraclinoid part of left carotid siphon
C. Arnold-Chiari malformation-herniation of cerebellar tonsils and part of
medulla through foramen magnum
D. Cerebellar atrophy/degeneration
E. Carbamazepine
F. Deformities of cervical spine
G. Diabetes mellitus
H. Encephalopathy
I. Ependymoma of posterior part of the fourth ventricle
J. Idiopathic
K. Insufficiency of basilar artery
L. Klippel-Feil anomaly-upward displacement of odontoid process into foramen
magnum
M. Meningioma extending into pontine cistern
N. Morphine poisoning
O. Multiple sclerosis (disseminated sclerosis)
P. Neurogenic muscular atrophy
Q. Platybasia (cerebellomedullary malformation syndrome)
Burde RM, Henkind P. Downbeat nystagmus. Surv Ophthalmol 1981; 25:263.
Chrousos GA. Downbeat nystagmus and oscillopsia associated with carbamazepine. Am J

Ophthalmol 1957; 103: 221-224.
Holmes GL, et al. Primary position upbeat nystagmus following meningitis. Ann
Monteiro ML, Sampaio CM. Lithium-induced downbeat nystagmus in a patient with

Arnold-Chiari malformation. Am J Ophthal 1993; 116:648-649.
ROTARY NYSTAGMUS (ROTARY OSCILLATORY MOVEMENT OF EYES)
1. Benign paroxysmal positional nystagmus-fast component toward lower ear

*2. Cerebellar disease-acute or chronic
3. Cerebrotendinous xanthomatosis
4. Encephalitis
5. Lesion of vestibular nuclei in floor of fourth ventricle associated with multiple
sclerosis, syringobulbia, or thrombosis of posteroinferior cerebellar artery or its branches
*6. Superior oblique myokymia-benign, intermittent, uniocular
7. Vestibular involvement (e.g., labyrinthitis, Meniere syndrome)
Leigh RJ, et al. Loss of ipsidirectional quick phases of torsional nystagmus with a
unilateral midbrain lesion. J Vestib Res 1993; 3:115-121.
Rosengart A, et al. Intermittent downbeat nystagmus due to vertebral artery compression.

Neurology 1993; 43: 216-218.
SEE-SAW NYSTAGMUS
One eye moves up as other eye moves down; in addition, there is torsion of eyes-
eye moving up intorts, and eye moving down extorts. This nystagmus probably is due to
lesions located in mesodiencephalic region, hypothalamus, and thalamus; it may be
associated with bitemporal hemianopsia and reduced vertical optokinetic nystagmus.
1. Brainstem vascular disease

*2. Chiasmal glioma
*3. Chromophobe adenoma of the pituitary gland involving the optic chiasm and third
ventricle
4. Congenital
*5. Craniopharyngioma, involving the optic chiasm and hypothalamus
6. Head injury with fracture of frontal
7. Idiopathic
9. Oligodendroglioma involving the pons and third ventricle
10. Postoperative after strabismus surgery
11. Retinitis pigmentosa
12. Septooptico dysplasia
13. Suprasellar epidermoid tumor involving optic chiasm and hypothalamus
14. Syringomyelia and syringobulbia
15. Toxoplasmosis of the brainstem
Fein JM, Williams RDB. See-saw nystagmus. J Neural Neurosurg Psychiatry 1969;
32:202-207.
Wilkins, 1985.
RETRACTION NYSTAGMUS
Spasmodic retraction of eyes can occur when an attempt is made to move them in
any direction; it is caused by lesions of midbrain, especially lesions in the vicinity of
aqueduct of Sylvius).
1. Arteriovenous aneurysm
2. Brucellosis (Bang disease)
3. Cysticercus cyst
4. Ependymoma
5. Koerber-Salus-Elschnig syndrome (sylvian aqueduct syndrome)
*6. Parinaud syndrome (paralysis of vertical movement)
7. Vascular lesions
Duke-Elder S, Scott GI. System of ophthalmology, Vol XII. St. Louis: CV Mosby, 1971.
MONOCULAR NYSTAGMUS
1. Horizontal
A. Lesions of optic nerve, chiasm, midbrain, or brainstem
B. Nervous system disease, such as multiple sclerosis, epidemic meningitis, and
congenital syphilis
C. Seizures
D. Superior oblique myokymia-benign, intermittent, uniocular
E. Spasmus nutans-most common cause in children
F. Tumors of brainstem
G. Unilateral amblyopia
H. Unilateral astigmatism or high refractive error
I. Unilateral opacity of the ocular media
2. Vertical
A. Multiple sclerosis
B. Myokymia of lower eyelid
C. Sleep
D. Spasmus nutans
E. Unilateral amblyopia
Farmer J, Hoyt CS. Monocular nystagmus in infancy and early childhood. Am J

Ophthalmol 1984; 98:504-509.
Gottlob I, et al. Signs distinguishing spasmus nutans from infantile nystagmus.

Ophthalmology 1990; 97: 1166.
Jacome DE, Fitzgerald R. Monocular ictal nystagmus. Arch Neural 1982; 39:653.
Smith JL, et al. Monocular vertical oscillations of amblyopia. J Clin Neurol 1982;
39:653.
PERIODIC ALTERNATING NYSTAGMUS

Periodic alternating nystagmus is central vestibular nystagmus with rhythmic jerk
type of nystagmus that undergoes phasic or cyclic changes in amplitude and direction.
1. Arnold-Chiari malformation
2. Cerebral trauma or fractured skull
3. Chiasmal lesion, such as craniopharyngioma
4. Chronic otitis media
5. Congenital
7. Encephalitis
8. Friedreich hereditary ataxia
9. Meningioma of tentorium cerebelli, cerebellar glioma, and cholesteatoma of the
cerebellopontine angle
10. Head trauma
11. Mesencephalic brainstem and cerebellar disease
13. Syringobulbia (Passow syndrome)
14. Syphilitic optic atrophy
15. Tumor of the corpus callosum
16. Vertebrobasilar artery insufficiency
17. Vestibular nuclei lesions
Davis DG, Smith JL. Periodic alternating nystagmus. Am J Ophthalmol 1971; 72:757-
762.
Wilkins, 1985.
POSITIONAL NYSTAGMUS
Positional nystagmus is nystagmus that appears or changes in form or intensity
after certain positional changes of the head indicate vestibular stimulation.
1. After general anesthesia

2. After head injury
acetophenazine baclofen carbromal
alcohol barbital carisoprodol
allobarbital bleomycin(?) carphenazine
alprazolam bromide cefaclor
amiodarone bromisovalum cefadroxil
amitriptyline broxyquinoline cefamandole
amobarbital bupivacaine cefazolin
amodiaquine butabarbital cefonicid
amoxapine butalbital cefoperazone
aprobarbital butallylonal ceforanide
aspirin butaperazine cefotaxime
auranofin butethal cefotetan
aurothioglucose calcitriol cefoxitin
aurothioglycanide carbamazepine cefsulodin
Bacille Calmette-Guerin carbinoxamine ceftazidime
(BCG) vaccine carbon monoxide ceftizoxime
ceftriaxone etidocaine methotrimeprazine
cefuroxime fenfluramine methoxamine
cephalexin flecainide methyl alcohol
cephaloglycin floxuridine methylpentynol
cephaloridine fluorouracil methylthiouracil
cephalothin fluphenazine methyprylon
cephapirin flurazepam metoclopramide
cephradine glutethimide metocurine iodide
chloral hydrate gold Au 198 metrizamide
chloramphenicol(?) gold sodium thiomalate mexiletine
chlordiazepoxide gold sodium thiosulfate midazolam
chloroform halazepam nalidixic acid
chloroprocaine hashish nifedipine
chloroquine heptabarbital nitrazepam
chlorpromazine hexethal nitrofurantoin
cholecalciferol hexobarbital norepinephrine
clemastine hydroxychloroquine nortriptyline
clomipramine ibuprofen oral contraceptives
clonazepam imipramine oxazepam
clorazepate influenza virus paramethadione
colistimethate vaccine(?) pemoline
colistin insulin penicillamine
cyclobarbital iodochlorhydroxyquin pentazocine
cyclopentobarbital iodoquinol pentobarbital
cytarabine iophendylate perhexiline
desipramine isoniazid pericyazine
diazepam ketarnine perphenazine
diethazine lidocaine phenelzine
digitalis(?) lithium carbonate phenobarbital
diiodohydroxyquin lorazepam phenylpropanolamine
dimethyl tubocurarine marijuana phenytoin
iodide meperidine piperacetazine
diphenhydramine mephenesin piperazine
diphenylhydantoin mephentermine polymyxin B
diphenylpyraline mephenytoin prazepam
disulfiram mephobarbital prilocaine
divalproex sodium mepivacaine primidone
doxepin meprobamate probarbital
doxylamine mesoridazine procaine
dronabinol metaraminol procarbazine
ergocalciferol methaqualone prochlorperazine
ergot(?) metharbital promazine
ethacrynic acid methdilazine promethazine
ethchlorvynol methitural propiomazine
ethopropazine methocarbamol propoxycaine
ethotoin methohexital protripty line
quinine thiopropazate tubocurarine
scopolamine thioproperazine urea(?)
secobarbital thioridazine urethan
sodium salicylate tobramycin valproate sodium
streptomycin tocainide valproic acid
talbutal tranylcypromine verapamil
temazepam triazolam vinbarbital
tetanus immune globulin trichloroethylene vinblastine(?)
tetanus toxoid trifluoperazine vincristine(?)
tetrahydrocannabinol triflupromazine vitamin A
thiamylal trimeprazine vitamin D
thiethylperazine trimethadione vitamin D2
thiopental trimipramine vitamin D3
thioperazine tripelennamine
4. Inner ear pathologic changes, including hemorrhage, inflammation, thrombosis,

emboli, circulatory and secretory conditions
5. "Normal" persons
6. Other causes include neuritis, meningitis, tumors, vascular anomalies, degeneration,
atrophy, syphilis, arteriosclerosis, hypertonia, vasomotor disturbance, allergic and toxic
conditions, cranial trauma, hemorrhage, emboli, or thrombosis
Fuijimoto M, et al. A study into the phenomenon of head-shaking nystagmus: its

presence in a dizzy population. J Otolaryngol 1993; 22:376-379.
Solmon SD. Positional nystagmus. Arch Otolaryngol 1969; 90:58-63.
OPTOKINETIC NYSTAGMUS
Normal physiologic nystagmus is obtained by watching moving targets; slow

components in direction targets are moving, and fast component move in the opposite
direction. Abnormal optokinetic nystagmus can be seen in the following:
1. Aberrant regeneration of third nerve-absent vertical optokinetic nystagmus, normal

horizontal optokinetic nystagmus
2. Internuclear palsies-horizontal targets bring out dissociation of ocular response
movements
3. Lesions of optic tract, geniculate body, temporal and occipital lobes show no
asymmetry of horizontal optokinetic responses
4. Lesions of parietal lobe give asymmetric horizontal optokinetic responses
5. Occipital lobe lesions with homonymous hemianopia and asymmetrical horizontal
optokinetic responses suggests a mass lesion extending into parietal lobe rather than a
vascular lesion
6. Parinaud syndrome-vertical optokinetic nystagmus with targets moving downward
enhances the retraction nystagmus seen on attempted upgaze.
7. Parkinsonism (shaking palsy)-vertical optokinetic nystagmus may be reduced
8. See-saw nystagmus-vertical optokinetic nystagmus may be reduced either upward or
downward (see p. 144)
9. Test for malingering in "blind" eye or eyes with normal optokinetic responses
Smith JL. Optokinetic nystagmus: its use in topical neuro-ophthalmologic diagnosis.

Springfield, IL: Charles C Thomas, 1963.
Wilkins, 1985.
SYNDROMES AND DISEASES ASSOCIATED WITH NYSTAGMUS
1. African eye-worm disease

2. Albers-Schonberg syndrome (osteosclerosis fragilis generalisata)
3. Albinism, ocular
4. Alexander disease
5. Anterior spinal artery syndrome
7. Arnold-Chiari syndrome (platybasia syndrome)
8. Arylsulfatase A deficiency syndrome
9. Babinski-Nageotte syndrome (medullary tegmental syndrome)
10. Bacterial endocarditis
12. Behçet syndrome (dermatostomatoophthalmic syndrome)
13. Behr disease (optic atrophy-ataxia syndrome)
14. Bielschowsky-Lutz-Cogan syndrome (internuclear ophthalmoplegia)
15. Bloch-Sulzberger disease (incontinentia pigmenti)*
16. Blocked nystagmus syndrome (nystagmus blockage syndrome)
18. Botulism
20. Caisson syndrome (bends)
21. Canavan disease (spongy degeneration of the white matter)
22. Cerebral palsy
23. Cestan-Chenais syndrome (combination of Babinski-Nageotte and Avellis syndrome)
24. Charcot-Marie- Tooth disease (progressive peroneal muscular atrophy)
25. CHARGE syndrome (colomba, heart disease, atresia, choanae, retarded growth and
retarded development or central nervous system anomalies, genital hypoplasia, and ear
anomalies or deafness syndrome)
stigmata)
27. Cherry-red spot myoclonus syndrome
28. Chromosome 6p12
29. Chromosome 18, partial deletion (long-arm) syndrome
31. Cogan syndrome (II) (oculomotor apraxia syndrome)
*32. Cone dysfunction syndrome (achromatopsia)
33. Costen syndrome (temporomandibular joint syndrome)
34. Craniocervical syndrome (whiplash syndrome)
35. Craniopharyngioma
36. Craniostenosis
37. Creutzfeldt-Jakob syndrome (spastic pseudosclerosis)
39. Curtius syndrome (ectodermal dysplasia with ocular malformations)
40. Cushing syndrome (II) (angle tumor syndrome)
41. Cytomegalic inclusion disease, congenital
44. Diencephalic syndrome (autonomic epilepsy syndrome) (Russell syndrome)
45. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
*46. Disseminated sclerosis (multiple sclerosis)
47. Down disease (mongolism, trisomy 21)
49. Eclampsia and preeclampsia
53. Epiphysial dysplasia, microcephaly, and nystagmus-autosomal recessive
54. Extreme hydrocephalus syndrome
55. Fanconi-Turler syndrome (familial ataxic diplegia)
56. Fetal hydantoin syndrome
57. Forsius-Eriksson syndrome (Aland disease)
58. François dyscephalic syndrome
59. Gangliosidosis (generalized gangliosidosis, infantile)
60. General fibrosis syndrome
62. Gorlin-Chaudhry-Moss syndrome
63. Guillain-Barré syndrome (acute infectious neuritis)
64. Hallervorden-Spatz syndrome (pigmentary degeneration of globus pallidus)
65. Hallgren syndrome (retinitis pigmentosa-deafness-ataxia syndrome)
66. Hand-Schüller-Christian syndrome (histiocytosis X)
67. Hanhart syndrome (recessive keratosis palmoplantaris)
68. Hartnup syndrome (niacin deficiency)
69. Hennebert syndrome (luetic-otitic-nystagmus syndrome)
70. Hermansky-Pudlak syndrome (oculocutaneous albinism and hemorrhagic diathesis)
71. Hurler syndrome (mucopolysaccharidoses I-H)
72. Hypervitaminosis D
73. Hypomelanosis of Ito syndrome (incontinentia pigmenti achromians)
74. Hypothyroidism (cretinism)
75. Hysteria
76. Infantile globoid cell leukodystrophy (Krabbe disease)
77. Infantile neuroaxonal dystrophy
79. Japanese River fever (typhus)
80. Jeune disease (asphyxiating thoracic dystrophy)
81. Kernicterus-high levels of bilirubin in the blood
82. Klippel-Feil syndrome (congenital brevicollis)
83. Koerber-Salus-Elschnig syndrome (sylvian aqueduct syndrome)
84. Kohn-Romano syndrome (blepharophimosis, ptosis, epicanthus inversus, telecanthus)
(Blepharophimosis syndrome)
85. Kugelberg-Welander syndrome (progressive proximal muscle atrophy)
adiposogenital syndrome)*
87. Leber congenital amaurosis syndrome (retinal aplasia)
89. Lenoble-Aubineau syndrome (nystagmus-myoclonia syndrome)
90. Lermoyez syndrome (form of Meniere disease)
92. Lockjaw (tetanus)
93. Louis-Bar syndrome (ataxia-telangiectasia syndrome)
94. Lowe disease (oculocerebrorenal syndrome)
96. Maple syrup urine disease
97. Marfan syndrome (acrachnadactyly-dystrophia mesodermalis congenita)
98. Marinesco-Sjögren syndrome (congenital spirocerebellar ataxia-congenital cataract-
oligophrenia syndrome)
98. Meniere syndrome (vertigo, tinnitus, nystagmus)
99. Meningococcemia (meningitis)
100. Mietens syndrome
101. Morning glory syndrome (optic nerve dysplasia, encephalocele)
102. Moyamoya disease (multiple progressive intracranial arterial occlusion)
103. Multiple lentigines syndrome (Leopard syndrome)
104. Naegeli syndrome (melanophoric nevus syndrome)
*105. Nystagmus, congenital
106. O'Donnell-Pappas syndrome (dominant foveal hypoplasia and presenile cataracts)*
optic nerve hypoplasia, coloboma
107. Papillon-Lerevre syndrome (hyperkeratosis palmoplantaris with periodontosis)
108. Parkinson disease
109. Passow syndrome (status dysraphicus syndrome)
110. Pelizaeus-Merzbacher disease (aplasia axialis extracorticalis congenita) - x-linked
111. Photomyoclonus, diabetes mellitus, deafness, neuropathy, and cerebellar
dysfunction- autosomal dominant
112. Poliomyelitis
113. Posthypoxic encephalopathy syndrome
114. Pyle disease (familial metaphyseal dysplasia)
115. Quincke disease (angioedema)
116. Rubella, congenital
117. Reimann syndrome (hyperviscosity syndrome)
120. Schilder disease (encephalitis periaxialis diffusa)
121. Seckel syndrome (bird-headed dwarf syndrome)*
122. Septooptic dysplasia (de Morsier syndrome)
124. Sorsby syndrome (hereditary macular coloboma syndrome)
125. Spastic paraplegia - x-linked
126. Split hand with congenital nystagmus, fundal changes, cataracts-autosomal
dominant
127. Stannus cerebellar syndrome (riboflavin deficiency)
128. Subclavian steal syndrome
129. Tay-Sachs disease (familial amaurotic idiocy)
130. Traumatic encephalopathy syndrome (punch-drunk syndrome)
131. Tremor, nystagmus, and duodenal ulcer-autosomal dominant
132. Tuomaala-Haapanen syndrome (similar to pseudohypoparathyroidism)
133. Vermis syndrome
134. Vertebral basilar artery syndrome
135. von Economo syndrome (encephalitis lethargica)
136. von Reuss syndrome (galactosemic syndrome)
137. Wagner syndrome (hyaloideoretinal degeneration)
138. Wallenberg syndrome (lateral bulbar syndrome)
140. Wernicke syndrome (superior hemorrhagic polioencephalopathic syndrome)
141. Wildervanck syndrome (cervicooculoacoustic syndrome)
142. Wilson disease (hepatolenticular degeneration)
144. Zellweger syndrome (cerebrohepatorenal syndrome of Zellweger)
Hayashi N, et al. Ocular histopathologic study of a patient with the T 8993-G point
mutation in Leigh's syndrome. Ophthalmology 2000; 107:1397-1402.
OCULOGYRIC CRISIS (SPASMODIC AND INVOLUNTARY DEVIATION OF

EYES, USUALLY UPWARD, LASTING FROM A FEW MINUTES TO
SEVERAL HOURS)
1. Cerebellar disease
acetophenazine flurazepam phencyclidine
alprazolam halazepam pimozide
alseroxylon haloperidol piperacetazine
amantadine hydroxychloroquine prazepam
amitriptyline imipramine prochlorperazine
amodiaquine influenza virus vaccine promazine
butaperazine levodopa promethazine
carbamazepine lithium carbonate propiomazine
carphenazine lorazepam protriptyline
chlordiazepoxide loxapine rauwolfia serpentina
chloroquine mesoridazine rescinnamine
chlorpromazine methdilazine reserpine
chlorprothixene methotrimeprazine syrosingopine
cisplatin metoclopramide temazepam
clonazepam metronidazole thiethylperazine
clorazepate midazolam thiopropazate
deserpidine nitrazepam thioproperazine
desipramine nortriptyline thioridazine
diazepam oxazepam thiothixene
diethazine pemoline triazolam
doxepin pentazocine trifluoperazine
droperidol perazine trifluperidol
ethopropazine pericyazine triflupromazine
fluphenazine perphenazine trimeprazine
3. Late manifestations of encephalitis

4. Lesions of fourth ventricle and cerebellum, especially lesions of the flocculus
6. Parkinsonism syndrome (shaking palsy)
8. Trauma
Burstein AH, Fullerton T. Oculogyric crisis possibly related to pentazocine. Ann

Pharmacotherapy 1993; 27:874-876.
OCULAR BOBBING
Both globes move synchronously in vertical plane by spontaneously and

intermittently dipping downward through an arc of a few millimeters and then return to
primary position; reverse ocular bobbing also has been described. Ocular bobbing differs
from vertical nystagmus by virtue of absence of a fast and a slow component in
movements; it is due to advanced pontine disease.
1. Acute organophosphate poisoning (Diazinon)
2. Associated with palatal myoclonus
*3. Encephalitis
4. Fibrocartilaginous embolism to the anterior spinal artery
5. Hypertensive pontine hemorrhage
6. Leigh encephalopathy (gangliosidosis GM type 3)
7. Locked-in syndrome
8. Phenothiazine and benzodiazepine poisoning (combined; reverse)
9. Ruptured giant distal posterior inferior cerebellar artery aneurysm
10. Thrombosis of basilar, middle cerebral, or vertebral arteries with posterior fossa
infarction
Hata S, et al. Atypical ocular bobbing in acute organophosphate poisoning. Arch Neurol
1986; 43:185-186.
Larmoude P, et al. Ocular bobbing: abnormal eye movement. Ophthalmology 1983;

187:161-165.
Osenbach RK, et al. Ocular bobbing with ruptured giant distal posterior interior
cerebellar artery aneurysm. Surg Neurol 1986; 25:149-152.
Tijssen CC, Terbruggen JP. Locked-in syndrome associated with ocular bobbing. Acta
Neurol Scand 1986; 73: 444-446.
PARALYSIS OF THIRD NERVE (OCULOMOTOR NERVE)

This type of paralysis includes ptosis, an inability to rotate the eye upward or
inward, a dilated unreactive pupil (iridoplegia), and paralysis of accommodation
(cycloplegia).
Extracted table paralysis of third nerve
1. Intracerebral
A. Lesion of red nucleus (Benedikt syndrome) - homolateral oculomotor paralysis
with contralateral intention tremor
B. Myasthenia Gravis and Mesencephalic Cavernous Angioma
C. Nuclear types-pareses of a single or a few extraocular muscles supplied by the
oculomotor nerve in one or both eyes; there mayor may not be pupillary disturbances
(mydriasis, sluggish pupillary reaction) and paresis of accommodation; in tumors within
or near the midbrain (pinealomas), there is a combination of isolated muscle pareses with
vertical gaze palsy, possibly a disturbance of convergence, and nystagmus retractorius
(Parinaud syndrome, sylvian aqueduct syndrome, pineal syndrome); includes Axenfeld-
Schurenberg syndrome (cyclic oculomotor paralysis), Bruns syndrome (postural change
syndrome), Claude syndrome (inferior nucleus ruber syndrome), congenital vertical
retraction syndrome, and Nothnagel syndrome (ophthalmoplegia-cerebellar ataxia
syndrome)
D. Occlusion of basilar artery-due to emboli especially but also to hemorrhage or
aneurysm
E. Recurrent third nerve palsy secondary to vascular spasm of migraine
F. Syndrome of cerebral peduncle (Weber syndrome)-homolateral oculomotor
paralysis and cross-hemiplegia
G. Tumors
2. Intracranial
A. Amebic dysentery
B. Aneurysm rupture at base of brain-third nerve paralysis, pain around the face
(fifth nerve), and headache
C. Botulism
D. Chickenpox
E. Craniopharyngioma
F. Dengue fever
G. Devic syndrome (optical myelitis)
H. Diphtheria
I. Encephalitis, acute
J. Hepatic failure
K. Hepatitis
L. Influenza
M. Lockjaw (tetanus)
N. Lymphoma
O. Malaria
P. Measles immunization
Q. Meningococcal meningitis
R. Multiple sclerosis (disseminated sclerosis)
S. Ophthalmic migraine
T. Periarteritis nodosa
U. Poliomyelitis
V. Polyneuritis because of toxins such as alcohol, lead, arsenic, and carbon
monoxide; dinitrophenol or carbon disulfide poisoning; or diabetes mellitus,
herpes zoster, or mumps
W. Rabies
X. Relapsing polychondritis
Y. Smallpox vaccination
Z. Subdural hematoma
AA. Syphilis (acquired lues)
BB. Temporal arteritis syndrome (Hutchinson-Horton-Magrath-Brown syndrome)
CC. Tuberculosis
3. Lesions affecting exit from cranial cavity
A. Cavernous sinus syndrome-paralysis of third, fourth, and sixth nerves with
proptosis
(1) Aneurysm (arteriovenous fistula syndrome)
(2) Carotid-cavernous fistula
(3) Cavernous sinus thrombosis
(4) Extension from lateral sinus thrombosis
(5) Extension of nasopharyngeal tumor
(6) Pituitary adenoma - lateral extension
(7) Tolosa-Hunt syndrome (painful ophthalmoplegia)
B. Superior orbital fissure syndrome-same as for cavernous sinus syndrome
except exophthalmos is less likely to occur and optic nerve involvement and
miotic pupil are more likely
(1) Aneurysm of internal carotid artery syndrome (foramen lacerum
syndrome)
(2) Occlusion of superior ophthalmic vein
(3) Skull fractures or hemorrhage
(4) Sphenoid sinus suppuration (sphenocavernous syndrome)
(5) Temporal syndrome (Gradenigo syndrome)
(6) Tumors, such as sphenoid ridge meningioma (Rochon-Duvigneaud
syndrome), nasopharyngeal tumor, metastatic carcinoma,
rhabdomyosarcoma, chordoma, and sarcoma
C. Orbital apex-involvement of third, fourth, sixth, and first division of fifth
cranial nerves and optic nerve proptosis is common
4. Other
A. Alber-Schonberg syndrome (marble bone disease, osteopetrosis)
B. Associated with aspirin poisoning
C. Congenital
D. Hodgkin disease
E. Lupus erythematosus (Kaposi-Libman-Sacks syndrome)
F. Myasthenia gravis (masquerade)
G. Passow syndrome (status dysraphicus syndrome)
H. Porphyria cutanea tarda
I. Sarcoid (Schaumann syndrome)
Harrison AR, Wirtschafter JD. Isolated inferior rectus paresis secondary to a

mesencaphalic cavernous angioma. Am J Ophthalmol 1999; 127, 5:617-620.
Holmes JM, et al. Pediatric third, fourth, and sixth nerve palsies: a population-based
study. Am J Ophthalmol 1999; 127:388-392.
Ing EB, et al. Oculomotor nerve palsies in children. J Pediatr Ophthal Strabismus 1992;
29:331-336.
Purvin V. Third cranial nerve palsy. In: Margo CE, ed. Diagnostic problems in clinical
ophthalmology. Philadelphia: WB Saunders, 1994:678.
Childhood Causes of Third Nerve (Oculomotor) Palsy

1. Trauma
2. Neoplasm
3. Undetermined
4. Ophthalmoplegic migraine
5. Postoperative cause
6. Meningitis/encephalitis
7. Subdural hematoma
8. Viral or post-upper respiratory tract infection
9. Varicella-zoster virus
10. Aneurysm
11. Orbital cellulitis
12. Sinus disease
13. Mesencephalic cyst
14. Cyclic oculomotor nerve palsy
15. Poison
Kodsi SR, Younge BR. Acquired oculomotor, trochlear, and abducent cranial nerve
palsies in pediatric patients. Am J Ophthalmol 1992; 114:568-574.
PARALYSIS OF FOURTH NERVE (TROCHLEAR NERVE)

This type of paralysis produces palsy of superior oblique muscle resulting in
limitation of downward movement of eye when it is in adducted position; it is frequently
associated with third cranial nerve palsy.
1. Intracerebral
A. Thrombosis of nutrient vessels, including median penetrating branch of basilar
artery to fourth nucleus
B. Hemorrhage in the roof of the midbrain
C. Aneurysm, including direct involvement by posterior cerebral and superior
cerebellar arteries
D. Tumors (rare if isolated fourth palsy)
(1) Primary
a. Gliomas, such as astrocytomas, ependymomas, and
medulloblastomas
b. Other primary tumors, including meningiomas, pinealomas,
craniopharyngiomas, and hemangiomas
(2) Unilateral trochlear nerve palsy
E. Metastatic lesions, such as those from the nasopharynx, rhabdomyosarcomas,
and neuroblastomas
F. Neonatal hypoxia
G. Nuclear type-trochlear paresis combined with a homolateral oculomotor
paresis, occasionally in association with vertical gaze palsies, convergence spasm
or convergence palsy, and pupillary disturbances seen in tumors of the roof of the
midbrain or pinealomas (pineal syndrome)
H. Claude syndrome (inferior nucleus ruber syndrome)
I. Passow syndrome (syringomyelia)
J. Inflammatory lesions, such as meningoencephalitis, cerebellitis, and abscess
K. Pseudotumor cerebri
2. Intracranial
A. Aneurysms, such as that of the posterior communicating artery or foramen
lacerum syndrome (aneurysm of internal carotid artery syndrome)
B. Hematomas, traumatic
C. Hydrocephalus
D. Meningitis, encephalitis, polyneuritis-diabetes mellitus, herpes zoster, multiple
sclerosis, myasthenia gravis, chickenpox, diphtheria, hydrophobia, Gradenigo
syndrome, influenza, malaria, poliomyelitis
E. Trauma
F. Tumors, including cerebellopontine angle tumor and pituitary adenoma
3. Lesions affecting exit from cranial cavity
A. Cavernous sinus syndrome (Foix syndrome)
B. Superior orbital fissure syndrome (Rochon-Duvigneaud syndrome)
C. Orbital apex syndrome (Rollet syndrome)
4. Orbital lesions
A. Fracture of superior orbital rim
B. Sinusitis
C. Operations on the frontal sinus in which there is trochlear displacement
D. Trochlear disturbance, such as in Paget disease or hypertrophic arthritis
E. Adherence syndrome-adhesions between the superior rectus and superior
oblique muscles
F. Abnormal insertion of superior oblique muscle or abnormal fascial attachments
G. Rochon-Duvigneaud syndrome (superior orbital fissure syndrome)
H. Idiopathic
Extracted Table Paralysis of fourth nerve
Feinberg AS, Newman NJ. Schwannoma in patients with isolated unilateral trochlear
Nerve palsy. Am J Ophthalmol 1999; 127:183-188.
Keane JR. Fourth nerve palsy: historical review and study of inpatients. Neurology 1993;
43:2439-2433.
Peterman SH, Newman NJ. Pituary macroadenoma manifesting as an isolated fourth

nerve palsy. Am J Ophthalmol 1999; 127, 2:235- 236.
Speer C, et al. Four cranial nerve palsy III pediatric patients with pseudotumor cerebri.
Am J Ophthalmol 1999; 127: 236-237.
Childhood Causes of Fourth Nerve (Trochlear) Palsy
1. Trauma
2. Neoplasm
3. Undetermined
5. Meningitis
6. Hydrocephalus
8. Viral infection
9. Aneurysm
10. Other
PSEUDOABDUCENS PALSY
1. Accommodative spasm
2. Blowout fracture (medial rectus entrapment)
3. Cross fixation (congenital esotropia)
5. Fibrosis of medial rectus
6. Horizontal gaze palsy (bilateral)-with or without contraction
7. Lack of effort involved in abducting a habitually adducted eye patch on other eye
differentiates from abducens palsy
8. Myasthenia gravis
9. Myositis
10. Orbital cellulitis (abscess)
11. Overambitious (large) resection of medial rectus
12. Thyroid myopathy (Graves disease, hyperthyroidism)
13. Unwillingness to cooperate-doll's head phenomenon (sudden passive turning of head)
differentiates from abducens palsy
Beyer-Machule C, von Noorden GK. Atlas of ophthalmic surgery, Vol 1: Lids, orbits,
Goldhammer Y. Pseudopalsy of the abducens nerve. In: Smith JL, ed. Neuro-
ophthalmology update. New York: Masson, 1977.
Extracted Table Paralysis of sixth nerve

Extracted Table Paralysis of third, fourth, and sixth cranial nerves
PARALYSIS OF SIXTH NERVE (ABDUCENS PALSY)

This type of paralysis produces palsy of the lateral rectus muscle with esotropia
increasing when the eye is moved laterally. The course of the sixth nerve makes it more
vulnerable to injury than other cranial nerves.
1. Intracerebral
A. Foville syndrome (Foville peduncular syndrome)
B. Gaucher disease (cerebroside lipidosis)
C. Hydrocephalus
D, Inflammatory lesions, such as meningoencephalitis, cerebellitis, and abscess
E. Lateral ventricular cyst
F. Leukemia
G. Millard-Gubler syndrome (abducens-facial hemiplegia alternans)
H. Mycoplasma pneumoniae
I. Nuclear aplasia - autosomal dominant
J. Platybasia (cerebellomedullary malformation syndrome)
K. Spontaneous subdural hematoma
L. Thrombosis or aneurysm of nutrient vessels to sixth nucleus-basilar artery
M. Tumors - intracranial, pontine glioma, or metastatic tumor from breast, thyroid
glands, or nasopharynx
(1) Primary
a. Gliomas, such as astrocytomas, ependymomas, and
medulloblastomas
b. Other primary tumors, including meningiomas, pinealomas,
craniopharyngiomas, and hemangiomas
(2) Metastatic lesions, such as those from the nasopharynx,
rhabdomyosarcomas, and neuroblastomas
N. Wernicke encephalopathy-thiamine deficiency in alcoholics with sixth nerve
palsy, paresis of horizontal conjugate gaze, nystagmus, ataxia, and Korsakoff
psychosis
2. Intracranial
A. Carotid artery aneurysm (foramen lacerum syndrome)
B. Cerebellopontine angle tumor, such as acoustic neuroma, producing unilateral
deafness, facial paralysis, diplopia, and papilledema
C. Chickenpox
D. Coccidioidomycosis
E. Congenital absence of sixth nerve
F. Cushing syndrome (II) (angle tumor syndrome)
G. Dandy-Walker syndrome (atresia of the foramen Magendie)
H. Diphtheria
I. Gradenigo syndrome - osteitis of petrous tip of pyramid following homolateral
mastoid or middle ear infection; facial pain (fifth nerve involvement)
J. Greig syndrome (ocular hypertelorism syndrome)
K. Hydrophobia (rabies)
L. Hydrocephalus (decreased intracranial pressure)
M. Increased intracranial pressure
N. Malaria
O. Massive pituitary adenoma
P. Measles
Q. Meningitis
R. Möbius syndrome (congenital paralysis of sixth and seventh nerves)
S. Neuritis because of diseases such as diabetes mellitus, herpes zoster,
poliomyelitis, lead or arsenic poisoning, multiple sclerosis, syphilis, brucellosis
T. Ophthalmoplegic migraine syndrome
U. Osteosarcoma
V. Passow syndrome (status dysraphicus syndrome)
W. Pseudotumor cerebri (Symonds syndrome)
X. Raymond syndrome (pontine syndrome)
Y. Relapsing polychondritis
Z. Skeletal dysplasia (mental retardation, abducens palsy)-x-linked
AA. Skull fractures-usually crush injury
BB. Spontaneous dissection of the internal carotid artery
CC. Subdural hematoma
DD. Trichinellosis
EE. Tumor extension as chordoma
FF. Vascular lesions, because of congenital aneurysm, arteriovenous fistulas,
diabetes, hypertension
GG. Water-soluble contrast myelography
3. Lesions affecting exit of sixth nerve from cranial cavity
A. Cavernous sinus syndrome (Foix syndrome)
B. Le Fort I maxillary osteotomy
C. Optic nerve sheath fenestration
D. Orbital apex lesion
E. Percutaneous thermal ablation of trigeminal nerve rootlet
F. Sphenocavernous syndrome
G. Sphenopalatine fossa lesion-loss of tearing and paresis of second division of
fifth nerve, most frequently because of malignant tumor
H. Superior orbital fissure syndrome
I. Tolosa-Hunt syndrome (painful ophthalmoplegia)
J. Transient in newborns
4. Other
A. Cluster headache
B. Cretinism (hypothyroid goiter)
C. Duane syndrome (retraction syndrome)
D. Engelmann syndrome (hereditary multiple diaphyseal sclerosis)
E. Following lumbar puncture, lumbar anesthesia, or Pantopaque injection for
myelography
F. Kahler disease (multiple myeloma)
G. Lupus erythematosus (Kaposi-Libman-Sacks syndrome)
H. Myasthenia gravis
I. Optic nerve sheath fenestration (rare)
J. Preeclampsia
K. Sarcoidosis
L. Secondary to immunization or viral illness
M. Toxic substances, such as arsenic, carbon tetrachloride, dichloroacetylene,
Dilantin, gold salts, isoniazid, nitrofuran, thalidomide, trichloroethylene, furaltadone
(Altafur), lithium
Mullaney PB, et al. Ophthalmic involvement as a presenting feature of nonorbital

childhood parameningeal embryonal rhabdomyosarcoma. Ophthalmology 200 1; 108:
179-182.
Rucker CW. The causes of paralysis of the third, fourth, and sixth cranial nerves. Am J
Childhood Causes of Sixth Nerve (Abducans) Palsy
1. Trauma
2. Neoplasm
3. Undetermined
4. Viral/benign
5. Gradenigo syndrome
6. Meningitis/encephalitis
7. Pseudotumor cerebri
8. Leukemia
9. Hydrocephalus
10. Arteriovenous malformation, brain
12. Miscellaneous
palsies in pediatric patients. Am J Ophthalmol 1992; 114, 5:568-574.
Childhood Causes of Third, Fourth, and Sixth Nerve Palsy

1. Trauma
2. Neoplasm
3. Undetermined
5. Meningitis
6. Hydrocephalus
8. Viral infection
9. Aneurysm
10. Other
ACUTE OPHTHALMOPLEGIA (ACUTE ONSET OF EXTRAOCULAR

MUSCLE PALSY)
1. Infranuclear
A. Aneurysm of internal carotid artery or circle of Willis
B. Trauma
(1) Orbital fracture
(2) Orbital hematoma
C. Orbital cellulitis secondary to acute paranasal sinusitis including
mucormycosis in a diabetic
D. Ophthalmoplegic migraine
F. Orbital pseudotumor
G. Orbital tumors
(1) Lymphoma
(2) Metastatic
2. Nuclear
A. Acute and subacute infections
(1) Infectious encephalitis
a. Viral encephalitis
(i) Anterior poliomyelitis
(ii) Encephalitis lethargica and other epidemic viral
encephalitides
(iii) Fisher syndrome (ophthalmoplegia, ataxia, areflexia)
(iv) Rabies
(v) Vaccinal encephalitis
(vi) Varicella, variola, measles, mumps, influenza,
infectious mononucleosis
(vii) Zoster
b. Organismal encephalitic infections
(i) Typhoid
(ii) Scarlet fever
(iii) Whooping cough
(iv) Gas gangrene
(v) Septicemia
(vi) Pneumonia
(vii) Typhus
(viii) Malaria
c. Acute central nervous system diseases
(i) Acute demyelinating diseases-acute disseminated
encephalomyelitis, acute multiple sclerosis
(ii) Neuritic infections
(a) Polyradiculoneuritis
(b) Epidemic paralyzing vertigo
(c) Acute infectious (rheumatic) polyneuritis
(d) Interstitial neuritis-meningitis, cranial sinusitis,
petrositis, nasal sinusitis, orbital periostitis, orbital
abscess
(iii) Widespread infections
(a) Meningovascular syphilis
(b) Mucormycosis (diabetes, immunosuppressed,
AIDS)
(c) Tuberculosis
(d) Torula and cryptococcosis
(iv) Toxic conditions
(a) Diphtheria
(b) Tetanus
(c) Botulism
(v) Allergic conditions
(a) Sarcoidosis syndrome (Schaumann syndrome)
(b) Recurrent multiple cranial nerve palsies
B. Metabolic diseases
(1) Deficiency diseases
a. Thiamine deficiency (Wernicke- Korsakoff syndrome)
b. Nicotinic acid deficiency-pellagra
c. Ascorbic acid deficiency-scurvy
(2) Diabetes
(3) Anemias
a. Primary anemia-leukemia
b. Secondary anemia (loss of blood)
(4) Exophthalmic ophthalmoplegia
(5) Porphyria
C. Poisoning such as lead, carbon monoxide, snake poisons, wasp stings, ergot,
sulfuric acid, phosphorus, triorthoceresylphosphate, and dichloroacetylene
D. Drugs, including the following:
acebutolol cisplatin gold Au 198
acetohexamide clomipramine gold sodium thiomalate
acetophenazine clonazepam gold sodium thiosulfate
adrenal cortex injection clorazepate griseofulvin
alcohol colchicine halazepam
aldosterone cortisone heptabarbital
allobarbital cyclobarbital hexachlorophene
amitriptyline cyclopentobarbital hexethal
amobarbital cytarabine hexobarbital
amodiaquine atenolol desipramine hydrocortisone
amoxapine desoxycorticosterone hydroxychloroquine
amphotericin B dexamethasone imipramine
aprobarbital dextrothyroxine indomethacin
aspirin diazepam(?) influenza virus vaccine
auranofin dibucaine insulin
aurothioglucose diethazine iodide and iodine
aurothioglycanide digitalis solutions and
barbital digitoxin compounds(?)
beclomethasone dimethyl tubocurarine iophendylate
betamethasone iodide isoniazid
botulin A toxin diphenhydramine ketoprofen
bupivacaine diphenylhydantoin labetalol
butabarbital diphtheria and tetanus levodopa
butalbital toxoids (adsorbed) levothyroxine
butallylonal diphtheria and tetanus lidocaine
butallylonal toxoids and pertussis liothyronine
butaperazine diphtheria toxoid liotrix
butethal (adsorbed) lorazepam
calcitriol(?) disulfiram measles and rubella
carbamazepine doxepin virus vaccine (live)
carisoprodol DPT vaccine measles virus vaccine
carphenazine ergocalciferol(?) measles, mumps, and
chloral hydrate ethambutol rubella virus vaccine
chlorambucil ethopropazine mephenesin
chlordiazepoxide etidocaine mephobarbital
chloroform fludrocortisone mepivacaine
chloroprocaine fluphenazine meprobamate
chloroquine fluprednisolone mesoridazine
chlorpromazine flurazepam metharbital
chlorpropamide glyburide methdilazine
methitural phenobarbital talbutal
methohexital phenylbutazone temazepam
methotrexate pindolol tetracaine
methotrimeprazine piperacetazine thiamylal
methoxiflurane piperazine thiethylperazine
methyl alcohol piperocaine thiopental
methyldopa poliovirus vaccine thiopropazate
methylene blue prazepam thioproperazine
methylprednisolone prednisolone thioridazine
metoclopramide prednisone thyroglobulin
metocurine iodide prilocaine thyroid trifluoperazine
metoprolol primidone tolazamide
metrizamide probarbital tolbutamide
midazolam procaine triamcinolone
mumps virus vaccine prochlorperazine trichloroethylene
(live) promazine triflupromazine
nadolol promethazine trimeprazine
nalidixic acid propiomazine trimethadione
naproxen propoxycaine trimipramine
nitrazepam proprandol tubocurarine
nitrofurantoin protriptyline vaccine (adsorbed)
nortriptyline quinacrine vinbarbital
oral contraceptives radioactive iodides(?) vinblastine
oxazepam rubella and mumps virus vincristine
oxyphenbutazone vaccine (live) vitamin A
paramethadione rubella virus vaccine vitamin D
paramethasone (live) vitamin D2
pentobarbital secobarbital vitamin D3
periciazine sodium salicylate
perphenazine phenytoin succinylcholine
E. Neoplasms and cysts

F. Trauma affecting the midbrain, base of the skull, and orbit
G. Vascular lesions as arteriosclerosis, hemorrhage and thrombosis in the
midbrain, subarachnoid, hemorrhage, aneurysms, congenitally dilated arteries,
giant-cell arteritis
H. Idiopathic-etiologic basis undetermined
Pacifici L, et al. Acute third cranial nerve ophthalmoplegia: possible pathogenesis from
alpha-II-interferon treatment. Ital J Neurol Sci 1993; 14:579-580.
CHRONIC OPHTHALMOPLEGIA (SLOW ONSET OF EXTRAOCULAR
MUSCLE PALSY)
1. Degenerative conditions
A. Amyotrophic lateral sclerosis-progressive bulbar palsy
B. Chronic progressive external ophthalmoplegia
C. Hereditary ataxias-Friedreich ataxia, Sanger-Brown ataxia
E. Syringomyelia (syringobulbia)
F. Thyroid myopathy (Graves disease)
2. Infective conditions
A. Diffuse sclerosis
B. Disseminated sclerosis (multiple sclerosis)
C. Syphilis
Duke-Elder S, Scott GI. System of ophthalmology, Vol XII. St. Louis: CV Mosby, 1971.
BILATERAL COMPLETE OPHTHALMOPLEGIA (BILATERAL PALSY OF

OCULAR MUSCLES, PTOSIS, WITH PUPIL AND ACCOMMODATION
INVOLVEMENT)
1. Arteriosclerotic hemorrhage and occlusion

2. Cerebellopontine angle tumors (Cushing syndrome II)
4. Fisher syndrome (ophthalmoplegia-ataxia areflexia syndrome)
5. Giant-cell arteritis (Hutchinson-Horton-Magath-Brown syndrome)
6. Kiloh-Nevin syndrome (ocular myeomyopathy)
7. Midbrain tumors
8. Multiple sclerosis (rare)
9. Mucormycosis
10. Ohaha syndrome (ophthalmoplegia, hypotonia, ataxia hypacusis, athetosis)
11. Orbital abscess
12. Parinaud syndrome (conjunctiva-adenitis syndrome)
13. Retrobulbar block complication
17. Trauma
18. Wernicke encephalopathies (thiamine deficiency)
19. Whipple disease (intestinal lipodystrophy)
Kaufman LM, et al. Invasive sinonasal polyps causing ophthalmoplegia, exophthalmos,
and visual field loss. Ophthalmology 1989; 96:1667-1672.
McKusick VA. Mendelian Inheritance in Man, 12th ed. Baltimore: Johns Hopkins
Hospital Press, 1998.
Sergott RC, et al. Simultaneous, bilateral diabetic ophthalmoplegia. Ophthalmology

1984; 91:18-22.
EXTERNAL OPHTHALMOPLEGIA (PARALYSIS OF OCULAR MUSCLES

INCLUDING PTOSIS WITH SPARING OF PUPIL AND ACCOMMODATION)
1. Abiotrophy-specific for one particular tissue, bilateral, symmetric

3. Aneurysm of internal carotid artery (foramen lacerum syndrome)
4. Bassen-Kornzweig syndrome (familial hypolipoproteinemia)
5. Bee sting
6. Chronic progressive external ophthalmoplegia
7. Congenital and familial
8. Diabetes mellitus (Willis disease)
9. Diphtheria
10. Epidemic encephalitis
11. Friedreich ataxia
12. Garcin syndrome (Schmincke tumor unilateral cranial paralysis)
13. Graves disease (hyperthyroidism)
14. Jacod syndrome (petrosphenoidal space syndrome)
15. Kearns-Sayre syndrome (ophthalmoplegic retinal degeneration syndrome)
16. Mumps
18. Myotonic dystrophy (Curschmann-Steinert syndrome)
19. Myositis
20. Nevus sebaceous of Jadassohn
21. Nothnagel syndrome (ophthalmoplegia-cerebellar ataxia syndrome)
22. Oculopharyngeal syndrome (progressive muscular dystrophy with ptosis and
dysphagia)
23. Olivopontocerebellar atrophy III (with retinal degeneration)-dominant
24. Ophthalmoplegia, progressive external, and scoliosis (horizontal gaze paralysis,
familial)-recessive
25. Pernicious anemia
26. Polyradiculoneuronitis (Guillain-Barré and Fisher syndromes)
27. Progressive facial hemiatrophy (Parry-Romberg syndrome)
28. Pseudotumor (orbital)
29. Refsum syndrome (heredopathia atactica polyneuritiformis syndrome)
31. Shy-Drager syndrome (orthostatic hypotension syndrome)
32. Shy-Gonatas syndrome (accumulation of lipids in muscles simulates gargoylism)
33. Tick paralysis (Lyme disease, Rocky Mountain spotted fever)
34. Vincristine-may have fifth and seventh nerve and peripheral neuropathies
35. Wernicke encephalopathies (beriberi, thiamine deficiency)
Fassati A, et al. Chronic progressive external ophthalmoplegia: a correlative study of

quantitative molecular data and histochemical and biochemical profile. J Neurol Sci
1994; 123:140-146.
Marsch SC, Schaefer HG. External ophthalmoplegia after total intravenous anaesthesia.
Anaesthesia 1994; 49: 525-527.
INTERNUCLEAR OPHTHALMOPLEGIA
This condition comprises paralysis of the medial rectus muscles on attempted
conjugate lateral gaze without other evidence of third nerve paralysis due to involvement
of medial longitudinal fasciculus. Jerk nystagmus of abducting eye and vertical
nystagmus, usually on upward gaze, may be present.
1. Bilateral
A. Arnold-Chiari malformation (cerebellomedullary malformation syndrome)
B. "Crack" cocaine
C. Fabry disease (glycosphingolipid lipidosis)
D. Inflammation, such as upper respiratory infection
E. Midbrain infarction
*F. Multiple sclerosis (disseminated sclerosis)
G. Myasthenia gravis (Erb-Goldflam syndrome)
H. Occlusive vascular disease
I. Oculocerebellar tegmental syndrome
J. Pontine hematoma
K. Syphilis (acquired lues)
L. Temporal arteritis
M. Vertebral basilar artery syndrome (whiplash injury)
N. Webino syndrome (wall-eyed exotropia bilateral internuclear
ophthalmoplegia)
O. Wernicke encephalopathy
2. Unilateral
A. Bielschowsky-Lutz-Cogan syndrome (internuclear ophthalmoplegia)
B. Cryptococcosis (torulosis)
C. Multiple sclerosis (disseminated sclerosis)
D. Myasthenia gravis (Erb-Goldflam syndrome)
E. Neuro-Behçet Disease
F. Tumors of the brainstem
*G. Vascular lesion-infarct of small branch of basilar artery
Atabay C, et al. Eales disease with internuclear ophthalmoplegia. Ann Ophthalmol 1992;
24:267-269.
Glaser JS. Neuro-ophthalmology, 3rd ed. Philadelphia: JB Lippincott, 1990.
Okuda B, et al. Bilateral internuclear ophthalmoplegia, ataxia, and tremor from a

midbrain infarction. Stroke 1993; 24:481-482.
Zee DS. Internuclear ophthalmoplegia: pathophysiology and diagnosis. Baillieres Clin

Neurol 1992; 1:455-470.
PAINFUL OPHTHALMOPLEGIA (PALSY OF OCULAR MUSCLES

WITH PAIN)
1. Adenocarcinoma metastatic to the orbit

2. Atypical facial neuralgia
3. Cavernous sinus syndrome (Foix syndrome)
4. Collier sphenoidal palsy
5. Diabetic ophthalmoplegia
6. Intracavernous carotid aneurysm
7. Myositis (orbital)
8. Nasopharyngeal tumor
10. Orbital abscess (mucormycosis-diabetes, immunosuppressed, AIDS)
11. Orbital apex sphenoidal syndrome (Rollet syndrome)
12. Orbital periostitis
13. Postherpetic neuralgia
14. Pseudotumor of orbit
15. Superior orbital fissure syndrome (Rochon-Duvigneaud syndrome, including superior
orbital fissuritis)
16. Temporal arteritis
17. Tic douloureux of the first trigeminal division
18. Tolosa-Hunt syndrome (inflammatory lesion of cavernous sinus)
Mannor GE, et al. Outcome of orbital myositis. Ophthalmology 1997; 104:409-414.
Sananman ML, Weintroub MI. Remitting ophthalmoplegia due to rhabdomyosarcoma.
Arch Ophthalmol 1971; 86: 6:459-461.
Extracted Table internuclear ophthalmoplegia
Extracted Table Painful Ophthalmoplegia
TRANSIENT OPHTHALMOPLEGIA (EXTRAOCULAR MUSCLE PARALYSIS

OF SHORT DURATION)
1. Cranial irradiation and intrathecal chemotherapy

2. Cyclic esotropia
3. Cyclic oculomotor palsy
4. Following internal carotid artery ligation for treatment of intracavernous giant
aneurysm
5. Lethargic encephalitis
6. Multiple sclerosis (disseminated sclerosis-usually the lateral rectus)
7. Myasthenia gravis (ocular, early)
8. Oculomotor nuclear complex infarction
9. Ophthalmoplegia migraine
10. Post lumbar puncture abducens palsy
12. Tabes dorsalis
13. Treatment of arteriovenous fistulas with Debrun balloon technique
Gadoth N, Liel Y. Transient external ophthalmoplegia in Wilson's disease. Metab

Pediatr. Ophthalmol 1980; 4:71-72.
Lepore FE, Nissenblatt MJ. Bilateral internuclear ophthalmoplegia after intrathecal

chemotherapy and cranial irradiation. Am J Ophthalmol 1981; 92:851-853.
Nakao S, et al. Transient ophthalmoplegia following internal carotid artery ligation for
treatment of intracavernous giant aneurysm. Surg Neurol 1982; 17:458-463.
PAINFUL OCULAR MOVEMENTS (PAIN WITH MOVEMENT OF THE EYES)
1. Bone-break fever (dengue fever) (rare)

2. Influenza
3. Myositis
A. "Collagen diseases"
B. Infectious myositis
C. Trichinosis
4. Orbital cellulitis
5. Orbital periostitis
Jampel RS, Fells P. Monocular elevation paresis caused by a central nervous system
lesion. Arch Ophthalmol 1968; 80:45.
POOR CONVERGENCE (INABILITY OF BOTH EYES TO FIXATE

SIMULTANEOUSLY ON A NEAR OBJECT)
1. Functional
A. Convergence insufficiency
B. Exophoria
C. Exotropia
D. Hysteria
E. Poor attention span
2. Organic
A. Brain lesion, to include bilateral occipital lobe lesions, superior colliculi, and
anterior internuclear ophthalmoplegia, such as in hemorrhage, trauma, or tumors
B. Dorsal midbrain syndrome
C. Encephalitis
D. Exophthalmic goiter-Möbius sign
E. Exophthalmos
F. Multiple sclerosis
G. Myotonic dystrophy
H. Narcolepsy
I. Poor visual acuity in one or both eyes
J. Postencephalitis
K. Syphilis and tabes
L. Third nerve paralysis (see p. 153)
M. Whiplash injury
alcohol cyclopentobarbital morphine
allobarbital dextroamphetamine opium
amobarbital dimethyl tubocurarine penicillamine
amphetamine iodide pentobarbital
aprobarbital diphenylhydantoin phenmetrazine
barbital floxuridine phenobarbital
bromide fluorouracil phenytoin
bromisovalum heptabarbital primidone
butabarbital hexethal probarbital
butalbital hexobarbital secobarbital
butallylonal mephobarbital talbutal
butethal methamphetamine thiamylal
carbamazepine metharbital thiopental
carbon dioxide methitural tubocurarine
chloral hydrate methohexital vinbarbital
cyclobarbital metocurine iodide
Wilkins, 1985.
SPASM OF CONVERGENCE
Spasm of convergence occurs with spasm of accommodation and miosis (i.e.,
spasm of the near reflex).
1. Encephalitis-accompanied with nystagmus

2. Hysteria-may be confused with lateral rectus palsy
3. Labyrinthine fistulas
4. Kenny syndrome
5. Oculogyric crisis in myasthenia gravis
6. Paralysis of horizontal gaze with compensatory spasm of near reflex
7. Parinaud syndrome (divergence paralysis)
8. Tabes dorsalis
9. Trauma
10. Wernicke syndrome (avitaminosis B1)
Feiler-Ofry V, et al. Lipoid proteinosis (Urbach-Wiethe Syndrome). Br J Ophthalmol

1979; 63:694-698.
Thompson RA, Zynde RH. Convergence spasm associated with Wernicke's

encephalopathy. Neurology 1969; 19: 711-712.
DIVERGENCE PARALYSIS
Divergence paralysis is defined as having a supranuclear cause with sudden onset
of comitant esotropia and uncrossed diplopia at distance, fusion at near (usually 1-2 m)
normal ductions and versions, and gross impairment of fusional amplitudes of
divergence.
*1. Brainstem lesions

A. Cerebellar cyst
B. Hemangioma
C. Tumors, such as cerebellar and acoustic neuromas and pontine glioma
2. Cerebral hemorrhage
3. Diazepam
4. Diphtheria
5. Dorsal midbrain syndrome
6. Epidemic encephalitis
7. Functional
8. Head injuries
9. Increased intracranial pressure
10. Influenza
11. Lead poisoning
13. Poliomyelitis
14. Syphilis
15. Unknown
16. Vascular disease
A. Diabetes mellitus
B. Hypertension
C. Occlusion of subclavian artery with reversal of flow in vertebral artery
D. Vertebral basilar insufficiency
Arai M, Fujii S. Divergence paralysis Neurology 1990; 237:45-46.
Brown SM, Iacuone JJ. Intact sensory fusion in a child with divergence paresis caused by
a pontine glioma. Am J Ophthalmol 1999; 128:528-530.
Krohel GB, et al. Divergence paralysis. Am J Ophthalmol 1982; 94:506.
Wilkins, 1985.
OCULOCARDIAC REFLEX
Bradycardia, nausea, and faintness occur and are dependent on trigeminal sensory
stimulation evoked by pressure on or within the eyeball or from sensory impulses by
stretching of ocular muscles.
1. Acute glaucoma
2. Anophthalmic socket
3. During ophthalmoscopy examination of premature infants
4. Exaggerated in epidemic encephalitis
5. Intermittent exophthalmos due to congenital venous malformations of the orbit
6. Intraocular injections
7. Orbital hematoma
*8. Pressure on globe
9. Retinal detachment operation
10. Severe injury to eye or orbit
*11. Traction on extraocular muscles including levator palpebrae superioris
Arnold RW, et al. Lack of global vagal propensity in patients with oculocardiac reflex.
Ophthalmology 1994; 101: 1347-1352.
Clarke WN, et al. The oculocardiac reflex during ophthalmoscopy in premature infants.
Am J Ophthalmol 1985; 99:649-651.
Ginsburg RN, et al. Oculocardiac reflex in the anophthalmic socket. Ophthalmic Surg
1992; 23:135-137.
Wilkins, 1985.
RETRACTION OF THE GLOBE (ON HORIZONTAL CONJUGATE GAZE)
*1. Duane syndrome (retraction syndrome)-cocontraction of horizontal rectus muscles,

lateral rectus, and both vertical muscles, or medial and inferior rectus muscles or fibrotic
lateral rectus
A. Acrorenoocular syndrome
B. Goldenhar syndrome
C. Hanhart syndrome
D. Isolated
E. Okihiro syndrome
F. Wildervanck syndrome (Klippel-Feil anomaly with Duane syndrome)
2. Fibrosis secondary to strabismus surgery
3. Medial wall fracture with incarceration of orbit contents-retraction of globe with
attempted abduction
4. Orbital mass
A. Dermoid cyst
B. Hemangioma
C. Lymphangioma
D. Osteofibroma
5. Retraction of convergent nonfixating eye associated with loss of conjugate lateral gaze
and occurrence of the near reflex on attempted lateral gaze
6. Thyroid myopathy
Holtz SJ. Congenital ocular anomalies associated with Duane's retraction syndrome, the
nevus of ota, and axial anisometropia. Am J Ophthalmol 1974; 74:729-731.
FORCED DUCTION TEST

The eyeball is moved away from the muscle being tested; this is accomplished by
grasping with a forceps the conjunctiva and episclera close to the limbus.
1. Supraduction - infraduction
A. Resistance
(1) Abnormal fascial or muscle attachments
(2) Congenital fibrosis syndrome
(3) Double elevator palsy
(4) Orbital floor fracture
(5) Orbital mass
(6) Thyroid myopathy of inferior rectus muscle
B. Unrestricted
(1) Elevator paresis
(2) Paresis of inferior superior rectus muscle
2. Supraduction in adduction
A. Brown superior oblique tendon sheath syndrome-resistance (see p. 132)
B. Paresis of inferior oblique muscle-unrestricted
3. Adduction
A. Resistance
(1) Chronic third nerve palsy with contracture lateral rectus
(2) Congenital fibrosis syndrome
(3) Duane retraction syndrome because of fibrosis of lateral rectus muscle
(4) Orbital mass
(5) Tight lateral rectus following excessive resection operation
(6) Thyroid myopathy
B. Unrestricted
(1) Extensive medial rectus recession
(2) Duane retraction syndrome because of central or peripheral
cocontraction of medial and lateral rectus on attempted adduction
4. Abduction
A. Resistance
(1) Abnormal fascial or muscle attachments including strabismus fixus
(2) Blowout fracture
(3) Chronic sixth nerve palsy with contracture medial rectus
(4) Myositis
(5) Orbital mass
(6) Thyroid myopathy
Tight medial rectus following excessive resection operation
B. Unrestricted
(1) Extensive lateral rectus recession
(2) Paralysis of lateral rectus muscle
Beyer-Machule C. von Noorden OK. Atlas of ophthalmic surgery, Vol I: Lids, orbits
DOUBLE ELEVATOR PALSY (APPARENT PARALYSIS OF GLOBE

ELEVATORS [I.E., SUPERIOR RECTUS AND INFERIOR OBLIQUE
MUSCLES])
1, Inferior rectus muscle restriction
A. Anomalous insertion of inferior rectus muscle
B. Blow-out fracture
C, Congenital orbital fibrosis syndrome
D. Thyroid ophthalmopathy
2. Monocular elevation paresis secondary to central nervous system lesions
A. Monocular elevation paresis
B. Skew deviation
3. Neurogenic or myogenic superior rectus muscle weakness
A. Myasthenia gravis (Erb-Goldflam syndrome)
B. Postoperative Berke-Motais surgery
C. Superior oblique tendon sheath syndrome (see p. 132)
D. Third cranial nerve palsy (see p. 153)
E. Trauma
Callahan M.A. Surgically mismanaged ptosis associated with double elevator palsy. Arch
Ophthalmol 1981:99:108.
OCULAR NEUROMYOTONIA (PAROXYSMAL MONOCULAR DEVIATIONS

ASCRIBABLE TO INVOLUNTARY CONTRACTION OF MUSCLES
INNERVATED BY THIRD, FOURTH, OR SIXTH CRANIAL NERVES)
1. Aneurysmal compression of third nerve

2. Chondrosarcoma
3. Cystic craniopharyngioma
4. Following radiation therapy
5. Rhabdomyosarcoma
Bateman DE, Saunders M. Cyclic oculomotor palsy. J Neurol Neurosurg Psychiatry

1983:46:451.
Lessell S, et al. Ocular neuromyotonia after radiation therapy. Am J Ophthalmol

1986:102:766-770.
EXTRAOCULAR MUSCLE ENLARGEMENT ON ORBITAL COMPUTERIZED

AND TOMOGRAPHIC SCAN
1. Diffuse
A. Acromegaly
B. Amyloidosis
*C. Graves disease as thyroid ophthalmopathy
*D. Infection
E. Parasitic infiltration
F. Trauma
G. Tumors, including pseudotumor
H. Vascular abnormalities as arteriovenous fistula
I. Collagen vascular disease
2. Focal
A. Cysticercosis
B. Hemorrhagic cyst
C. Primary or metastatic carcinoma
D. Trichinella
Albert OM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Saunders, 1994:1881-2095.
Howard GR, et al. Orbital dermoid located within lateral rectus muscle. Ophthalmology
1994;101:767-778.
Kaltreider SA, et al. Destructive cysts of the maxillary sinus affecting the orbit. Arch
Ophthalmol 1988; 106: 1398-1402.
Maurrello JA, Flanagan JC. Management of orbital inflammatory disease: a protocol.

Surv Ophthalmol 1984; 29: 104-116.
5
Conjunctiva
CONTENTS
Cellular responses 183
Purulent conjunctivitis 185
Acute mucopurulent conjunctivitis 186
Chronic mucopurulent conjunctivitis 186
Membranous conjunctivitis 187
Pseudomembranous conjunctivitis 187
Ophthalmia neonatorum 191
Acute follicular conjunctivitis 192
Chronic follicular conjunctivitis 193
Cicatricial conjunctivitis 194
Angular conjunctivitis 194
Conjunctival disorders associated with dermatologic disorders 195
Conjunctival disorders associated with genital disorders 196
Congestion of conjunctiva 197
Ciliary flush 201
Conjunctival aneurysms, varicosities, tortuousities, and telangiectasis 201
Conjunctival sludging and segmentation 202
Conjunctival edema 202
Conjunctival xerosis 205
Bitot spots 206
Subconjunctival hemorrhage 206
Tumors of the conjunctiva 211
Conjunctival cysts 212
Limbal mass 212
Large, flat, fleshy lesions of palpebral conjunctiva 213
Chronic or recurrent ulcers of the conjunctiva 214
Phlyctenular keratoconjunctivitis 214
Pigmentation of the conjunctiva 215
Discoloration of conjunctiva 216
Symblepharon 217
Conjunctival concretions 218
Lesions of caruncle 218
CELLULAR RESPONSES
1. Basophilic reaction-significant only when seen in large numbers; immunoglobulin E

(Ig-E) mediated allergic conditions; key role in asthma, atopy, and hypersensitivity
responses; present in vernal (eosinophils) and giant papillary conjunctivitis (GPC)
conjunctivitis.
2. Eosinophilic reaction-GPC (giant papillary conjunctivitis); Charcot-Leyden crystals
are more prominent than intact eosinophils in chronic allergy; parasitic conjunctivitis.
*A. Vernal conjunctivitis-characteristic with fragmentation of eosinophil
B. Hay fever conjunctivitis-rarely fragmentation of eosinophil
C. Allergic conjunctivitis from various drugs, cosmetics, and other antigens
D. Atropine sensitivity-not present when eserine or pilocarpine is used
3. Mononuclear reaction
A. Viral disease-l00% without secondary infection; usually lymphocytic
*(1) Epidemic keratoconjunctivitis-adenovirus type 8
(2) Pharyngoconjunctival fever-adenovirus type 3
*(3) Herpetic keratoconjunctivitis
(4) Acute follicular conjunctivitis of Beal
B. Chronic ocular infections
4. Neutrophilic reaction-early stage of severe viral conjunctivitis
A. All bacteria but two-Neisseria catarrhalis and Haemophilus duplex (Morax-
Axenfeld diplobacillus)
B. Viruses of the family Chlamydiaceae [trachoma inclusion conjunctivitis
(TRIC) agent)
(1) Trachoma
*(2) Inclusion conjunctivitis
(3) Lymphogranuloma venereum
C. Fungal disease
(1) Streptothrix conjunctivitis secondary to canaliculitis
(2) Nocardial corneal ulcers
(3) Monilial corneal ulcers
D. Unknown cause
*(1) Erythema multiforme (Stevens-Johnson syndrome)
(2) Conjunctivitis of Reiter disease
E. Vernal conjunctivitis-eosinophilic and neutrophilic reaction
F. Epidemic keratoconjunctivitis and herpetic keratoconjunctivitis have a shift
from mononuclear to polymorphonuclear reaction when a membrane is formed
5. Plasma-cell reaction-trachoma-especially with spontaneous rupturing of follicles;
chlamydial conjunctivitis
6. Epithelial changes
A. Keratinization of conjunctival epithelial cells
*(1) Alkali bum
(2) Vitamin A deficiency
(3) Exposure
(4) Cicatrization (such as pemphigoid and Stevens-Johnson syndrome)
(5) Keratoconjunctivitis sicca-partially keratinized epithelial cells, specific
(6) Epithelial plaque
(7) Superior limbic keratoconjunctivitis (SLK)
B. Large, multipointed epithelial cells
(1) Characteristic of viral infection
*(2) Most often found in herpetic keratitis
C. Intracellular granules
(1) Pseudoinclusion bodies-extension of nuclear material into cytoplasm
(2) Intracellular-free green pigment in cytoplasm-present in persons with dark
complexion
(3) Intracellular-free blue granules-present in cytoplasm in about 12% of
normal individuals
(4) Sex chromatin-present in nuclei of females only
7. Cellular inclusions
A. Trachoma and inclusion conjunctivitis have identical inclusions-basophilic,
cytoplasmic (Halberstaedter-Prowazek)
*B. Molluscum contagiosum-eosinophilic, cytoplasmic (Henderson/Patterson)
C. Lymphogranuloma venereum-eosinophilic
*D. Herpes simplex and herpes zoster-eosinophilic, internuclear (Lipschütz)
E. Measles-multinucleated giant cells with eosinophilic internuclear inclusion
bodies and cytoplasmic, eosinophilic masses
*F. Chickenpox -eosinophilic, internuclear
G. Smallpox-eosinophilic, cytoplasmic
Brinser JH, Burd EM. Principles of diagnosis ocular microbiology. In: Tabbara KF,
Hyndiuk RA, eds. Infections of the eye. Boston: Little, Brown, 1986:73-92.
Fedukowicz HB. External infections of the eye: bacterial, viral, mycotic, 3rd ed. New
York: Appleton-Century- Crofts, 1984.
Soparkar, Charles NS, et al. Arch Opthalmol 1997; 115:34-38.
Spencer WH. Ophthalmic pathology, Vol I. Philadelphia: WB Saunders, 1985.
PURULENT CONJUNCTIVITIS
Purulent conjunctivitis is characterized as violent acute conjunctival
inflammation, great swelling of lids, copious secretion of pus, and a marked tendency to
corneal involvement and even possible loss of the eye.
1. Gram-positive group
A. Bacillus of Doderlein (Lactobacillus sp.)
B. Listeria monocytogenes
*C. Pneumococcus
D. Staphylococcus
*E. Streptococcus
2. Gram-negative group
A. Aerobacter aerogenes
B. Enterobacteriaceae
C. Escherichia coli
*D. Haemophilus influenzae biotype III
E. Klebsiella pneumoniae
F. Moraxella lacunata
*G. Neisseria gonorrhoeae
H. Neisseria meningitidis
I. Proteus species
J. Pseudomonas species
K. Serratia marcescens
3. Vaccinia virus
4. Fungus
A. Actinomyces species
B. Candida species
*C. Nocardia species
5. Wiskott - Aldrich syndrome- x-linked
Fedukowicz HB. External infections of the eye: bacterial, viral, mycotic, 3rd ed. New
York: Appleton-Century-Crofts, 1984.
Snead JW, et al. Listeria monocytogenes endophthalmitis. Am J Ophthalmol 1977;

84:337-344.
ACUTE MUCOPURULENT CONJUNCTIVITIS

This type of conjunctivitis is epidemic pink eye, marked hyperemia and a
mucopurulent discharge, which tends toward spontaneous recovery.
1. Gram-positive group
*A. Pneumococcus
B. Staphylococcus-eyelid lesions and punctate staining of the lower cornea may
occur
2. Gram-negative group
*A. Haemophilus aegyptius (Koch-Weeks bacillus)
B. H. influenzae
3. Associated with exanthems and viral infections
A. German measles (Greig syndrome)
B. Measles (rubeola)
C. Mumps
*D. Reiter syndrome (conjunctivourethrosynovial syndrome)
E. Scarlet fever
4. Fungus
A. Candida albicans
B. Leptothrix
5. Lyell disease-toxic epidermal necrolysis or scalded-skin syndrome
7. Sjögren syndrome (secretoinhibitor syndrome)
8. Etiology obscure in many cases
Fedukowicz HB. External infections of the eye: bacterial, viral, and mycotic, 3rd ed.
New York: Appleton-Century-Crofts, 1984.
Okumoto M, Smolin G. Pneumococcal infections of the eye. Am J Ophthalmol 1974;
77:346-352.
CHRONIC MUCOPURULENT CONJUNCTIVITIS (MUCOPURULENT

DISCHARGE, MODERATE HYPEREMIA WITH A CHRONIC COURSE)
1. Infective element-lids or lacrimal apparatus

A. Monilia species
B. Morax-Axenfeld diplobacillus (angular conjunctivitis)
*C. Pneumococcus
D. Pubic lice
E. Staphylococcus
F. Streptothrix foersteri
2. Allergic-cosmetic
3. Irritative
A. Associated infections or irritation of lids, lacrimal apparatus, nose, or skin
B. Deficiency of lacrimal secretions
C. Direct irritants-foreign body, mascara, dust, wind, smog, insecticides,
chlorinated water, and many others
D. Exposure-ectropion, facial paralysis, exophthalmos, and others
E. Eyestrain
F. Metabolic conditions-gout, alcoholism, or prolonged digestive disturbances
G. Overtreatment by drugs-antibiotics, miotics, mydriatics
New York: Appleton-Century- Crofts, 1984.
Geyer O, et al. Phenylephrine prodrug. Ophthalmology 1991; 98:1483.
Okumoto M, Smolin G. Pneumococcal infections of the eye. Am J Ophthalmol 1974;

77:346-352.
MEMBRANOUS CONJUNCTIVITIS
Exudate permeates epithelium to such an extent that removal of membrane is
difficult and a raw bleeding surface results. Membranous conjunctivitis can lead to
symblepharon, ankyloblepharon, and entropion with trichiasis.
1. Chemical irritants
A. Acids, such as acetic or lactic
*B. Alkalis, such as ammonia or lime
C. Metallic salts, such as silver nitrate or copper sulfate
2. Corynebacterium diphtheriae
3. Ligneous conjunctivitis-chronic, cause unknown
4. Pneumococcus
5. Streptococcus
6. Uncommon-actinomyces, glandular fever, measles, Neisseria catarrhalis, variola,
Pseudomonas aeruginosa, herpes simplex, Leptothrix, and epidemic keratoconjunctivitis
(type adenovirus)
Fedukowicz HB. External infections of the eye: bacterial. Viral, and mycotic, 3rd ed.
PSEUDOMEMBRANOUS CONJUNCTIVITIS
In pseudomembranous conjunctivitis, the fibrin network is easily peeled off,
leaving the conjunctiva intact; it forms on the conjunctiva.
1. Bacteria
A. C. diphtheriae
*B. Gonococcus
*C. Meningococcus
D. Pneumococcus
E. Staphylococcus
*F. Streptococcus
G. Uncommon-H. aegyptius, H. influenzae, N. catarrhalis, Pseudomonas
aeruginosa, E. coli, Bacillus subtilis, Shigella, Bacillus faecalis alcaligenes, Salmonella
paratyphi B, Mycobacterium tuberculosis, and Treponema pallidum
2. Viral
*A. Epidemic keratoconjunctivitis (type adenovirus)
*B. Herpes simplex
C. Herpes zoster
D. Reiter syndrome (conjunctivourethrosynovial syndrome)
E. Vaccina
3. Fungal- C. albicans
*4. Allergic-vernal conjunctivitis
5. Toxic
*A. Stevens-Johnson syndrome can be caused by drugs, including the following:
acetaminophen amidone ampicillin
acetanilid aminosalicylate(?) antipyrine
acetazolamide aminosalicylic acid(?) aprobarbital
acetohexamide amithiozone aspirin
acetophenazine amobarbital auranofin
allobarbital amodiaquine aurothioglucose
allopurinol amoxicillin aurothioglycanide
barbital clindamycin methdilazine
belladonna cloxacillin methicillin
bendroflumethiazide cyclobarbital methitural
benzathine penicillin G cyclopentobarbital methohexital
benzthiazide cyclothiazide methotrimeprazine
bromide danazol methsuximide
bromisovalum demeclocycline methyclothiazide
butabarbital dichlorphenamide methylphenidate
butalbital dicloxacillin metolazone
butallylonal diethazine minocycline
butaperazine diphenylhydantoin minoxidil
butethal doxycycline moxalactam
captopril enalapril nafcillin
carbamazepine erythromycin naproxen
carbenicillin ethopropazine oxacillin
carbromal ethosuximide oxyphenbutazone
carisoprodol ethotoin oxytetracycline
carphenazine ethoxzolamide paramethadione
cefaclor fenoprofen penicillin
cefadroxil fluphenazine pentobarbital
cefamandole furosemide perazine
cefazolin gentamicin pericyazine
cefonicid glyburide perphenazine
cefoperazone gold Au 198 phenacetin
ceforanide gold sodium thiomalate phenobarbital
cefotaxime gold sodium thiosulfate phenoxymethyl
cefotetan heptabarbital penicillin
cefoxitin hetacillin phensuximide
cefsulodin hexethal phenylbutazone
ceftazidime hexobarbital phenytoin
ceftizoxime hydrabamine penicillin V piperacetazine
ceftriaxone hydrochlorothiazide plproxen
cefuroxime hydroflumethiazide polythiazide
cephalexin hydroxychloroquine potassium penicillin G
cephaloglycin ibuprofen potassium penicillin V
cephaloridine indapamide potassium phenethicillin
cephalothin indomethacin potassium
cephapirin isoniazid phenoxymethyl
cephradine lincomycin primidone
chloroquine mephenytoin pro barbital
chlorothiazide mephobarbital procaine penicillin G
chlorpromazine meprobamate prochlorperazine
chlorpropamide mesoridazine promazine
chlortetracycline methacycline promethazine
chlorthalidone metharbital proparacaine
cimetidine methazolamide propiomazine
propranolol sulfamethizole thiethylperazine
quinethazone sulfamethoxazole thiopental
quinine sulfamethoxypyridazine thiopropazate
rifampin sulfanilamide thioproperazine
secobarbital sulfaphenazole thioridazine
smallpox vaccine sulfapyridine tolazamide
sodium salicylate sulfasalazine tolbutamide
sulfacetamide sulfathiazole trichlormethiazide
sulfachlorpyridazine sulfisoxazole trifluoperazine
sulfacytine sulindac triflupromazine
sulfadiazine sulthiame trimeprazine
sulfadimethoxine talbutal trimethadione
sulfamerazine tetracycline vancomycin
sulfameter thiabendazole vinbarbital
sulfamethazine thiamylal
*B. Benign mucous membrane pemphigoid can be caused by drugs, including the
following:
carbamazepine isoniazid practolol
carbimazole methimazole propylthiouracil
diphenylhydantoin methsuximide streptomycin
ethosuximide methylthiouracil trimethadione
griseofulvin paramethadione
hydralazine phensuximide
C. Lyell disease (toxic epidermal necrolysis or scalded-skin syndrome) can be

caused by drugs, including the following:
acetaminophen benzthiazide cloxacillin
acetanilid betamethasone cortisone
acetazolamide bismuth oxychloride cyclobarbital
acid bismuth sodium bismuth sodium cyclopentobarbital
tartrate bismuth sodium cyclophosphamide
adrenal cortex injection bismuth sodium tartrate cyclothiazide
aldosterone busulfan dapsone
allobarbital butabarbital demeclocycline
amobarbital butalbital desoxycorticosterone
amoxapine butallylonal dexamethasone
amoxicillin butethal dichlorphenamide
ampicillin carbamazepine dicloxacillin
antipyrine carbenicillin diltiazem
aprobarbital carbimazole diphenylhydantoin
aurothioglucose chlorambucil doxepin
aurothioglycanide chlorothiazide doxycycline
barbital chlortetracycline erythromycin
bendroflumethiazide chlorthalidone ethambutol
benzathine penicillin G clomipramine ethotoin
ethoxzolamide minocycline sulfacetamide
fludrocortisone nafcillin sulfachlorpyridazine
fluprednisolone nitrofurantoin sulfadiazine
gold Au 198 oxacillin sulfadimethoxine
gold sodium thiomalate oxyphenbutazone sulfamerazine
heptabarbital oxytetracycline sulfameter
hetacillin paramethadione sulfamethazine
hexethal paramethasone sulfamethizole
hexobarbital penicillamine sulfamethoxazole
hydrabamine pentobarbital sulfamethoxypyridazine
penicillin V phenobarbital sulfanilamide
hydrochlorothiazide phenoxymethyl sulfaphenazole
hydrocortisone penicillin sulfapyridine
ibuprofen phenylbutazone sulfasalazine
indapamide phenylbutazone sulfathiazole
indomethacin phenytoin sulfisoxazole
isoniazid piroxicam sulindac
kanamycin poliovirus vaccine talbutal
mechlorethamine polythiazide tetracycline
melphalan potassium penicillin G thiabendazole
mephenytoin potassium penicillin V thiamylal
mephobarbital potassium phenethicillin thioglycollate
meprednisone prednisolone thiopental
methacycline prednisone triamcinolone
metharbital primidone trichlormethiazide
methazolamide probarbital triethylene-melamine
methicillin procaine penicillin G triglycollamate
methitural procarbazine trimethadione
methohexital quinethazone trimipramine
methotrexate secobarbital uracil mustard
methyclothiazide smallpox vaccine vinbarbital
methylprednisolone sodium salicylate
metolazone streptomycin
D. Pemphigus vulgaris
E. Hereditary epidermolysis bullosa
6. Chemical irritants
A. Acids, such as acetic or lactic
*B. Alkalis, such as ammonia or lime
C. Metallic salts, such as silver nitrate or copper sulfate
D. Vegetable and animal irritants
7. Acute graft-versus-host disease
8. Foot-and-mouth disease
9. Koch-Weeks bacillus
10. Ligneous conjunctivitis-chronic, cause unknown
11. Lipoid proteinosis (Urbach-Wiethe disease)
*12. Superior limbic keratoconjunctivitis
13. Traumatic or operative healing of wounds
14. Wegner granulomatosis
Barthelemy H, et al. Lipoid proteinosis with pseudomembranous conjunctivitis. J Am

Acad Dermatol 1986; 14: 367-371.
OPHTHALMIA NEONATORUM (CONJUNCTIVTIS OCCURRING IN

NEWBORNS)
* 1. Chemical conjunctivitis, such as from silver nitrate instillation

*2. Chlamydial trachomatis
3. Bacteria
A. Gram positive
(1) C. diphtheriae
(2) Staphylococcus aureus
(3) Staphylococcus epidermidis
(4) Streptococcus group D
(5) Streptococcus pneumoniae
(6) Streptococcus viridans
B. Gram negative
(1) Coliform bacillus, such as E. coli
(2) Enterobacter cloacae
(3) Haemophilus influenzae
(4) Haemophilus parainfluenzae
(5) K. pneumoniae
(6) Meningococcus
(7) Mima polymorpha - gram negative
(8) N. gonorrhoeae and N. catarrhalis
(9) Neisseria organisms
(10) Pneumonococcus
(11) Proteus mirabilis
(12) P. aeruginosa
(13) Pseudomonas pyocyanea
(14) S. marcescens
4. Virus
A. Herpes simplex
B. Streptococcus viridans
C. Coxsackie A
D. TRIC virus
5. Other
A. Acinetobacter species
B. Branhamella catarrhalis
C. C. albicans
D. Citrobacter feundi
E. Clostridium perfringes
F. Inclusion blennorrhea
G. Listeriosis (L. monocytogenes)
H. Moraxella species
I. Mycoplasma organisms
J. Peptococcus prevotii
K. Propionibacterium species
L. Trichomonas vaginalis
Cohen KL, McCarthy LR. Haemophilus influenzae ophthalmia neonatorum. Arch

Rapoza PA, et al. Epidemiology of neonatal conjunctivitis. Ophthalmology 1986; 94:461.
Stenson S, et al. Conjunctivitis in the newborn: observations on incidence, cause, and

prophylaxis. Ann Ophthalmol 1981:13:329.
ACUTE FOLLICULAR CONJUNCTIVITIS LYMPHOID FOLLICLES

(COBBLESTONING) OF CONJUNCTIVA WITH RAPID ONSET
*1. Inclusion conjunctivitis-adult inclusion conjunctivitis (AIC) (begins 2 days after

exposure to organism, may be bilateral, no systemic symptoms, and a unilateral or
bilateral preauricular node is often present.
*2. Adenovirus conjunctivitis-EKC has been reported worldwide from virus serotypes
(the most common are 8, 11, and 19); pharyngoconjunctival fever (PCF) is usually
caused by serotypes 3, 4, and 7.
A. Pharyngoconjunctival fever-usually because of type adenovirus; common in
swimming-pool epidemics in the summer and fall
B. Epidemic keratoconjunctivitis because of adenovirus type (rarely occurs in
children)
*3. Primary herpetic keratoconjunctivitis-conjunctival reaction may be follicular or
pseudomembranous
4. Newcastle disease (fowlpox) conjunctivitis-usually seen in poultry handlers,
veterinarians (caused by a paramyxovirus: single-stranded RNA virus that causes
respiratory infections)
5. Influenza virus A
6. Herpes zoster
*7. Cat-scratch fever (Parinaud oculoglandular syndrome)-fever caused by two types of
rickettsia: Rochalimaea henselae and Afipia felis)
8. Echovirus keratoconjunctivitis
9. Trachoma (sometimes)
10. Bacterial Streptococcus, Moraxella, and Treponema organisms
11. Mesantoin use
12. Chlamydia epizootic (feline pneumonitis)
13. Ophthalmomyiasis
14. Acute hemorrhagic conjunctivitis
15. Neonatal inclusion conjunctivitis
16. Unknown types-a case that resists etiologic classification is encountered occasionally;
it is probable that other viruses occasionally produce acute follicular conjunctivitis
17. Associated with regional adenitis
A. Angelucci syndrome (critical allergic conjunctivitis syndrome)
B. Anoxic overwear syndrome
C. Benjamin-Allen syndrome (brachial arch syndrome)
D. Floppy eyelid syndrome
*E. Giant papillary conjunctivitis syndrome
*F. Inclusion conjunctivitis in adults-acute mucopurulent follicular inflammation,
persisting as long as several months, sometimes with scarring
G. Syndrome of Beal-transient unilateral disease, usually resolving in weeks
Allansmith MR. The eye and immunology. St. Louis: CV Mosby, 1982.
Kowalski RP, Harwick JC. Incidence of Moraxella conjunctival infection. Am J

Ophthalmol 1986; 101:437-440.
Uchio E, et al. Acute follicular conjunctivitis caused by adenovirus type 34. Am J

Ophthalmol 1990; 128:680-686.
CHRONIC FOLLICULAR CONJUNCTIVITIS (LYMPHOID FOLLICLES

COBBLES TONING) OF CONJUNCTIVA WITH LONG-TERM COURSE)
1. Chronic follicular conjunctivitis-Axenfeld's type (orphan's) frequently found in

institutionalized children; almost asymptomatic; long duration (to months or longer); no
keratitis; cause unknown
*2. Chronic follicular conjunctivitis, toxic type
A. Bacterial origin, such as that due to a diplobacillus or other microorganism
B. Drugs, including the following:
acyclovir diatrizoate meglumine F3T
adenine arabinoside and sodium framycetin
amphotericin B diisopropyl gentamicin
apraclonidine fluorophosphate homatropine
atropine dipivefrin hyaluronidase
carbachol DPE idoxuridine
clonidine echothiophate isoflurophate
demecarium eserine ketorolac tromethamine
methscopolamine pilocarpine sulfisoxazole
neomycin scopolamine trifluorothymidine
neostigmine sulfacetamide trifluridine
physostigmine sulfamethizole vidarabine
3. Chronic follicular conjunctivitis with epithelial keratitis; differentiated from Axenfeld

type by shorter duration (to months) and by epithelial keratitis involving upper third of
cornea; epidemic in schools; can be transmitted by mascara pencil; cause unknown
4. Ectodermal syndrome (Rothmund syndrome)
5. Folliculosis-associated general lymphoid hypertrophy
*6. Molluscum contagiosum conjunctivitis
7. Neurocutaneous syndrome (ectodermal dysgenesis)
8. Parinaud syndrome-chronic fever and regional lymphadenopathy, frequently cat-
scratch fever
*9. Postoperative penetrating keratoplasty or cataract surgery sutures
10. Sebaceous carcinoma with papillary conjunctivitis
11. Trachoma-stages to 3
12. Use of hard and soft contact lens
*14. Use of ocular prostheses
15. With generalized lymphadenopathy
Cunningham ET, Koehler JE. Ocular bartonellosis. Am J Ophthalmol 2000; 130:340-349.
Gloor P, et al. Sebaceous carcinoma presenting as a unilateral papillary conjunctivitis.

Am J Ophthalmol 1999; 124, 4:458-459.
Mondino BJ, et al. Allergic and toxic reactions in soft contact lens wearers. Surv
CICATRICIAL CONJUNCTIVITIS (SCARRING OF CONJUNCTIVA)
*1. General: a postinfectious type of membranous conjunctivitis such as C. diphtheriae,

streptococcal conjunctivitis, autoimmune or presumably autoimmune sarcoidosis,
scleroderma, Stevens-Addison, pemphigoid, lichen planus, atopic blepharoconjunctivitis,
miscellaneous causes and linear IgA dermatosis.
2. Upper lid
A. Trachoma
3. Lower lid
*A. Acne rosacea (ocular rosacea)
B. Chemical (especially alkali)
C. Chlamydia organisms (psittacosis-lymphogranuloma group)
D. Chronic cicatricial conjunctivitis-occurs in the elderly; has a chronic course;
may have concurrent skin and mucous membrane lesions
*E. Congenital syphilis
F. Dermatitis herpetiformis
G. Epidemic keratoconjunctivitis
H. Epidermolysis acuta toxica (Lyell syndrome)
I. Epidermolysis bullosa
*J. Erythema multiforme (Stevens-Johnson disease)
K. Erythroderma ichthyosiforme
L. Exfoliative dermatitis
M. Fuchs-Lyell syndrome
N. Hydroa vacciniforme
O. Impetigo
P. Lamellar ichthyoses
*Q. Ocular pemphigoid
R. Paraneoplastic lichen planus
S. Radium bums
T. Reiter syndrome (conjunctivourethrosynovial syndrome)
U. Sjögren syndrome (secretoinhibitor syndrome)
V. Staphylococcal granuloma
W. Syphilis (acquired lues)
X. Systemic scleroderma (progressive systemic sclerosis)
Y. Vaccinia
*4. Drugs
A. Demecarium bromide
*B. Echothiophate iodide
C. Idoxuridine
D. Penicillamine
E. Pilocarpine
F. Practolol
G. Thiabendazole
H. Timolol
*I. Topical ocular epinephrine
Chan LS, et al. Ocular cicatricial pemphigoid occurring as a sequela of Stevens-Johnson

syndrome. JAMA 1991; 266:1543-1546.
Chiou AG, et al. Management of conjunctival cicatrizing diseases and severe ocular
surface dysfunction. Surv Ophthalmol 1998; 43:19-46.
Cruz AV, et al. Eyelid abnormalities in lamellar ichthyoses. Ophthalmology 2000;

107:1895-1898.
Hahn JM, et al. Cicatrizing conjunctivitis associated with paraneoplastic lichen planus.
Am J Ophthalmol 2000; 129: 98-99.
Hoang-Xuan T, et al. Epidermolysis bullosa acquisita diagnosed by direct

immunoelectron microscopy of the conjunctiva. Ophthalmology 1997; 104:1414-1420.
Hoang-Xuan T, et al. Pure ocular cicatricial pemphigoid. Ophthalmology 1999; 106:355-

362.
ANGULAR CONJUNCTIVITIS (INFLAMMATION AT ANGLE OF EYE,

USUALLY LATERAL)
1. C. albicans
*2. M. lacunata (Morax-Axenfeld diplobacillus)
3. Stannus cerebellar syndrome (riboflavin deficiency)
4. S. aureus
Scheie HG. Textbook of ophthalmology, 10th ed. Philadelphia: WB Saunders, 1986.
CONJUNCTIVAL DISORDERS ASSOCIATED WITH DERMATOLOGIC

DISORDERS
1. Dermatoses
A. Acanthosis nigricans
B. Acne rosacea
C. Acrodermatitis chronica atrophicans
D. Acrodermatitis enteropathica
*E. Atopic eczema dermatitis
F. Diffuse cutaneous mastocytosis
G. Erythroderma exfoliativa (Wilson-Brocq disease)
H. Ichthyosis
I. Keratosis follicularis
J. Keratosis follicularis spinulosa decalvans
K. Lichen planus
L. Pityriasis rubra pilaris; lichen acuminatus
M. Porokeratosis
N. Psoriasis vulgaris
O. Seborrhea
P. Xeroderma pigmentosum
2. Mucocutaneous eruptions
*A. Behçet disease (dermatostomatoophthalmic syndrome)
B. Benign mucous membrane pemphigoid
C. Dermatitis herpetiformis (Duhring-Brocq disease)
*D. Erythema multiforme (Stevens-Johnson disease)
E. Epidermolysis bullosa
F. Hydroa vacciniforme (recurrent summer eruption)
G. Pemphigus-vulgaris, vegetans, foliaceus
H. Pyostomatitis vegetans
I. Reiter disease (polyarthritis enterica)
Wilson LA. External diseases of the eye. New York: Harper & Row, 1979.
CONJUNCTIVAL DISORDERS ASSOCIATED WITH GENITAL DISORDERS

1. Bacteria
A. Bacteroides species
B. Calymmatobacterium granulomatis (granuloma inguinale)
C. E. coli
D. Haemophilus ducreyi
E. Haemophilus vaginalis
F. Mimeae species
G. Mycobacterium leprae
H. M. tuberculosis
*I. N. gonorrhoeae
J. Proteus species
*K. P. aeruginosa
L. Staphylococcus species
M. Streptococcus species
2. Fungi
A. Candida species
B. Other
3. Viruses
A. Cytomegalovirus
*B. Herpes virus hominis 2
*C. Molluscum contagiosum virus
D. Rubella
E. Varicella zoster
F. Verruca virus
4. Spirochetes
A. T. pallidum
5. Chlamydiae
A. Chlamydia lymphogranuloma
*B. Chlamydia oculogenitalis
C. Unclassified Chlamydia from Reiter disease
6. Parasites
A. Beetles
B. Fly larvae
C. Moths
D. Phthirus pubis
E. T. vaginalis
Roy FH. Ocular syndromes and systemic diseases. 3rd ed. Philadelphia: Lippincott
Vergnami RJ, Smith RS. Reiter syndrome in a child. Arch Ophthalmol 1974:91:165-166.
CONGESTION OF CONJUNCTIVA (NONINFECTIOUS HYPEREMIA OF THE

CONJUNCTIVA)
* 1. Acute lupus erythematosus (Kaposi-Libman-Sacks syndrome)

2. Alcoholism
*3. Allergic conjunctivitis, such as contact with cosmetics or plastic
4. Avitaminosis
5. Carcinoid syndrome
*6. Carotid-cavernous fistula or arteriovenous aneurysm
7. Cavernous sinus thrombosis (Foix syndrome)
8. Conjunctivitis caused by air pollution (smog, dust, or smoke)
*9. Drugs causing conjunctival hyperemia, including the following:
acetohexamide aurothioglucose cefazolin
acetylcholine aurothioglycanide cefonicid
acyclovir barbital cefoperazone
adrenal cortex injection beclomethasone ceforanide
adsorbed belladonna cefotaxime
alcohol bendroflumethiazide cefotetan
aldosterone benzalkonium cefoxitin
allobarbital benzthiazide cefsulodin
allopurinol betamethasone ceftazidime
alprazolam bischloroethylnitrosourea ceftizoxime
alseroxylon (BCND) ceftriaxone
alseroxylon brimonidine tartrate cefuroxime
amithiozone bupivacaine cephalexin
amobarbital compounds butalbital cephaloglycin
and pertussis vaccine butallylonal cephaloridine
antazoline butethal cephalothin
apraclonidine carbachol cephapirin
aprobarbital carmustine cephradine
aspirin cefaclor chloral hydrate
atropine cefadroxil chloramphenicol
auranofin cefamandole chlordiazepoxide
chloroform emetine lithium carbonate
chloroprocaine ephedrine lorazepam
chlorothiazide erythromycin maprotiline
chlorpropamide ether measles and rubella
chlortetracycline ethotoin virus
chlorthalidone ethyl biscoumacetate measles virus vaccine
chrysarobin etidocaine (live)
cimetidine F3T medrysone
cisplatin fenoprofen meperidine
clindamycin fludrocortisone mephentermine
clindamycin fluorescein mephenytoin
clofibrate fluorometholone mephobarbital
clonazepam fluprednisolone mepivacaine
clorazepate flurazepam meprednisone
colchicine flurbiprofen mercuric oxide
colloidal silver gentamicin metaraminol
cortisone glyburide methacholine
cyclobarbital gold Au metharbital
cyclopentobarbital gold sodium thiomalate methimazole
cycloserine gold sodium thiosulfate methitural
cyclosporine griseofulvin methocarbamol
cyclothiazide halazepam methohexital
cytarabine heparin methoxamine
deferoxamine heptabarbital methoxsalen
deserpidine hexethal methyclothiazide
desoxycorticosterone hexobarbital methyldopa
dexamethasone homatropine methylprednisolone
dextran hydralazine methylthiouracil
dextrothyroxine hydrochlorothiazide metolazone
diacetylmorphine hydrocortisone metoprolol
diatrizoate meglumine hydroflumethiazide metronidazole
and diazepam ibuprofen mianserin
dicumarol idoxuridine (IDU) midazolam
diethylcarbamazine indapamide minoxidil
difumarate indomethacin morphine
diltiazem interferon moxalactam
dimercaprol iodide and iodine mumps virus vaccine
diphenadione solutions (live)
diphtheria and tetanus iothalamate meglumine naproxen
toxoids and iothalamic acid nifedipine
disodium clodronate ketoprofen nitrazepam
disodium etidronate levothyroxine nitromersol
disodium pamidronate lidocaine norepinephrine
emedastine lincomycin olopatadine HCI
disopyramide liothyronine opium
doxorubicin liotrix oxazepam
oxprenolol radioactive iodides sulfathiazole
oxyphenbutazone ranitidine sulfisoxazole
oxyphenonium rauwolfia serpentina syrosingopine
paramethasone rescinnamine talbutal
pentazocine reserpine temazepam
pentobarbital rifampin tetanus immune globulin
phenacetin rubella and mumps virus tetanus toxoid
phenindione rubella virus vaccine tetracycline
phenobarbital (live) thiabendazole
phenoxybenzamine scopolamine thiacetazone
phenprocoumon secobarbital thiamylal
phenylbutazone sildenafil citrate thimerosal
phenylephrine silver nitrate thiopental
phenylmercuric acetate silver protein thiotepa
phenylmercuric nitrate sodium chloride thyroglobulin
pilocarpine sodium salicylate thyroid
piroxicam streptomycin tolazamide
piroxicam sulfacetamide tolazoline
polythiazide sulfachlorpyridazine tolbutamide
practolol sulfacytine triamcinolone
prazepam sulfadiazine triazolam
prazosin sulfadimethoxine trichlormethiazide
prednisolone sulfamerazine trichloroethylene
prednisone sulfameter trifluridine
prilocaine sulfamethazine trioxsalen vaccine (live)
primidone sulfamethizole vancomycin
probarbital sulfamethoxazole verapamil
procaine sulfamethoxypyridazine vidarabine
propoxycaine sulfanilamide vinbarbital
propranolol sulfaphenazole vitamin A
propylthiouracil sulfapyridine
quinethazone sulfasalazine
10. Gout (hyperuricemia)

11. Hormone deficiency (estrogenic)
12. Hypothyroidism
13. Irritative follicular conjunctivitis (see p. 193)
*A. Chemical conjunctivitis because of drugs, including the following:
acenocoumarol anisindione benzthiazide
acetaminophen antazoline butabarbital
acetanilid antipyrine butalbital
acetohexamide aprobarbital butallylonal
allobarbital aspirin butethal
allopurinol barbital carbamazepine
alprazolam bendroflumethiazide carbimazole
amobarbital benzalkonium cefaclor
cefadroxil diphtheria and tetanus methylthiouracil
cefamandole disopyramide metolazone
cefazolin doxorubicin metronidazole
cefonicid emetine mianserin
cefoperazone ephedrine midazolam
ceforanide ethotoin minoxidil
cefotaxime ethyl biscoumacetate morphine
cefotetan fenoprofen moxalactam
cefoxitin flurazepam mumps virus vaccine
cefsulodin flurbiprofen (live)
ceftazidime halazepam naproxen
ceftizoxime heparin nifedipine
ceftriaxone heptabarbital nitrazepam
cefuroxime hexethal opium
cephalexin hexobarbital oxazepam
cephaloglycin hydralazine oxprenolol
cephaloridine hydrochlorothiazide oxyphenbutazone
cephalothin hydroflumethiazide oxyphenonium
cephapirin ibuprofen pentazocine
cephradine indapamide pentobarbital
chloramphenicol indomethacin phenacetin
chlordiazepoxide interferon phenindione
chlorothiazide iodide and iodine phenobarbital
chlorpropamide solutions and phenprocoumon
chlortetracycline compounds phenylbutazone
chlorthalidone iothalamate phenylephrine
chrysarobin iothalamic acid piroxicam
cimetidine ketoprofen polythiazide
cisplatin lithium carbonate practolol
clofibrate lorazepam prazepam
clonazepam maprotiline prazosin
clorazepate measles and rubella primidone
colloidal silver virus vaccine (live) probarbital
cyclobarbital measles virus vaccine propranolol
cyclopentobarbital (live) propylthiouracil
cycloserine meglumine and sodium quinethazone
cyclosporine meperidine radioactive iodides
cyclothiazide mephenytoin ranitidine
cytarabine mephobarbital rubella and mumps virus
dextran metharbital vaccine (live)
diazepam methimazole rubella virus vaccine
dicumarol methitural (live)
diethylcarbamazine methocarbamol secobarbital
diltiazem methohexital silver nitrate
dimercaprol methyclothiazide silver protein
diphenadione methyldopa sodium salicylate
streptomycin sulfaphenazole tolazamide
sulfacetamide sulfapyridine tolbutamide
sulfachlorpyridazine sulfasalazine toxoids and pertussis
sulfacytine sulfathiazole vaccine
sulfadiazine sulfisoxazole triazolam
sulfadimethoxine talbutal trichlormethiazide
sulfamerazine temazepam trichloroethylene
sulfameter tetanus immune globulin verapamil
sulfamethazine tetanus toxoid vinbarbital
sulfamethizole tetracycline vitamin A
sulfamethoxazole thiamylal warfarin
sulfamethoxypyridazine thiopental
sulfanilamide thiotepa
B. Topical drugs that are hypotonic, hypertonic, or in which the pH is above or

below 6.9 or a drug degradation causing chemical irritation
C. Toxic conjunctivitis because of drugs such as miotics or cycloplegics
D. Vegetable irritants (e.g., caster bean)
*14. Malignant lymphoma
15. Ophthalmic vein thrombosis
16. Photosensitive conjunctivitis
*17. Polycythemia vera (Vaquez disease)
19. Vascular changes
A. Facial paralysis (see p. 66-68)
*B. Hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber disease)
C. Petechial hemorrhage of conjunctiva (see p. 206)
Collins JF. Handbook of clinical ophthalmology. New York: Masson, 1982.
CILIARY FLUSH
Ciliary flush involves circumcorneal congestion and congestion of ciliary vessels
immediately surrounding the cornea; individual vessels are not seen; color is violaceous;
redness fades toward the fornices; and vessels do not move with conjunctiva.
1. Corneal disease, such as with inflammations and erosions

2. Glaucoma, especially acute glaucoma
3. Iridocyclitis
4. Iris irritation, such as with corneal foreign bodies
5. Iritis
Vaughan D, et al. General ophthalmology, 14th ed. Los Altos, CA: Lange Medical, 1995.
CONJUNCTIVAL ANEURYSMS, VARICOSITIES, TORTUOUSITIES, AND
TELANGIECTASIS
1. Local causes
*A. Acne rosacea
B. Chronic congestive glaucoma
C. Delayed mustard gas keratitis
D. Idiopathic anomaly
E. Irradiation of the eye
F. Long-standing ocular inflammation
*G. Metastatic primary tumor
*H. Pterygium
*I. Underlying choroidal or ciliary body melanomas
2. Systemic causes
A. Acquired immunodeficiency syndrome (AIDS)
B. Arteriosclerosis
C. Associated with familial amyloidotic polyneuropathy, type 1
D. Ataxic telangiectasia (Louis-Bar syndrome)
*E. Degos syndrome (malignant atrophic papulosis)
F. Diabetes
G. Dysproteinemia as in Waldenström macroglobulinemia, cryoglobulinemia, and
multiple myeloma
H. Endangiitis obliterans
I. Fabry disease (diffuse angiokeratosis)
J. Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease)
K. Hypertension
L. Klippel-Trenaunay-Weber syndrome (angioosteohypertrophy syndrome)
M. Normal individuals
*N. Pulmonary insufficiency
O. Reimann syndrome (hyperviscosity syndrome)
P. Renal failure
Q. Rheumatic fever or rheumatic heart disease
R. Scleroderma (progressive systemic sclerosis)
S. Sturge-Weber syndrome (meningocutaneous syndrome)
*T. Syphilis (acquired lues)
U. Varicose veins-generalized
Ando E, et al. Ocular microangiopathy in familial amyloidotic polyneuropathy, type 1.

Graefes Arch Clin Exp Ophthalmol 1992; 230:1-5.
Baumann S, et al. Conjunctival microvasculopathy and Kaposi's sarcoma in patients with

AIDS. AIDS 1994; 8: 134-135.
CONJUNCTIVAL SLUDGING AND SEGMENTATION
1. Local
A. Aging
B. Hypothermia
C. Sympathetic irritation
D. Vasodilator drugs that are applied locally
2. Systemic or hyperviscosity with increase in serum proteins
A. Cryoglobulinemia
B. Hyperglobulinemia
C. Hypertension
D. Macroglobulinemia (Waldenström syndrome)
*E. Multiple myeloma (Kahler disease)
*F. Sickle cell disease (Herrick syndrome)
Lu LM, et al. Sjögren's syndrome and benign hyperglobulinemic purpura of

Waldenström. Ann Ophthalmol 1981; 13: 1285-1287.
Maisel JM, et al. Multiple myeloma presenting with ocular inflammation. Ann
Ophthalmol 1987; 19:170-174.
CONJUNCTIVAL EDEMA (CHEMOSIS)
1. Acquired blockage of orbital lymphatics following orbital surgery (lateral orbitotomy)

or because of erysipelas or lymphogranuloma venereum
*2. Chronic hereditary lymphedema (Nonne-Milroy-Meige disease)
*3. Drugs, including the following:
acetohexamide antimony lithium benzalkonium
acetophenazine thiomalate benzathine penicillin G
acetyldigitoxin antimony potassium betamethasone
actinomycin C tartrate bleomycin
adrenal cortex injection antimony sodium brinzolamide
albuterol tartrate bupivacaine
aldosterone antimony sodium butabarbital
allobarbital thioglycollate butacaine
amantadine antipyrine butalbital
aminopterin aprobarbital butallylonal
aminosalicylate aspirin butaperazine
aminosalicylic acid auranofin butethal
amobarbital aurothioglucose cactinomycin
amodiaquine aurothioglycanide captopril
amphotericin B barbital carbachol
and compounds benoxinate carbamazepine
carbenicillin danazol griseofulvin
carphenazine dapsone heptabarbital
cefaclor daunorubicin hetacillin
cefadroxil demecarium hexachlorophene
cefamandole demeclocycline hexamethonium
cefazolin desipramine hexethal
cefonicid deslanoside hexobarbital
cefoperazone desoxycorticosterone hydrabamine penicillin
ceforanide dexamethasone hydralazine
cefotaxime dextrothyroxine hydrocortisone
cefotetan diacetylmorphine hydroxychloroquine
cefoxitin diatrizoate meglumine ibuprofen
cefsulodin and idoxuridine
ceftazidime dibucaine IDU
ceftizoxime dicloxacillin imipramine
ceftriaxone diethazine iodide and iodine
cefuroxime diethylcarbamazine solutions
cephalexin digitoxin iron dextran
cephaloglycin dionin isoflurophate
cephaloridine dorzolamide isosorbide
cephalothin doxepin isotretinoin
cephapirin doxorubicin ketoprofen
cephradine doxycycline lanatoside C
chloral hydrate dromostanolone levodopa
chlorambucil dyclonine levothyroxine
chloramphenicol echothiophate lidocaine
chlorhexidine emetine lithium carbonate
chlorisondamine enalapril mannitol
chloroprocaine epinephrine maprotiline
chloroquine ergonovine mecamylamine
chlorpromazine ergotamine medrysone
chlorpropamide erythromycin melphalan
chlortetracycline ethopropazine mephobarbital
chrysarobin etidocaine mepivacaine
cisplatin etretinate meprednisone
clofibrate F3T mercuric oxide
clomipramine floxuridine mesoridazine
cloxacillin fludrocortisone metharbital
cocame fluorouracil methdilazine
colistin fluoxymesterone methitural
colloidal silver fluphenazine methohexital
cortisone fluprednisolone methotrimeprazine
cyclobarbital gitalin methyldopa
cyclopentobarbital glyburide methylergonovine
cyproheptadine gold Au 198 methylpentynol
dactinomycin gold sodium thiomalate methylprednisolone
methysergide pipobroman stibocaptate
metoclopramide poliovirus vaccine stibophen
metrizamide polynoxylin B streptomycin
metronidazole potassium penicillin G succinylcholine
mianserin potassium penicillin V sulindac
mild silver protein potassium phenethicillin suramin
minocycline practolol talbutal
mitomycin prazosin testolactone
moxalactam prednisolone testosterone
nafcillin prednisone tetracaine
naproxen prilocaine tetracycline
neomycin primidone tetraethylammonium
nitromersol probarbital thiamylal
nortriptyline procaine thiethylperazine
oral contraceptives procaine penicillin G thimerosal
ouabain prochlorperazine thiopental
oxacillin promazine thiopropazate
oxprenolol promethazine thioproperazine
oxyphenbutazone propiomazine thioridazine
oxytetracycline propoxycaine thiotepa
paramethasone protriptyline thyroid
pentobarbital quinacrine tolazamide
pentolinium radioactive iodides tolbutamide
perazine rifampin triamcinolone
pericyazine rubella virus vaccine trifluoperazine
perphenazine (live) triflupromazine
phenacaine sanguinarine trimeprazine
phenazine secobarbital trimethaphan
phenobarbital silicone trimethidinium
phenylbutazone silver nitrate urokinase
phenylephrine silver protein vidarabine
pilocarpine sodium vinbarbital
piperacetazine sodium vinblastine
piperazine antimonylgluconate
piperocaine sodium salicylate
4. Glandular fever
5. Hypersensitivity-local topical allergies
*6. Increased bulk of orbital contents-orbital tumors, cysts, or endocrine exophthalmos
7. Local inflammatory conditions
*A. Cerebral cavity-acute meningitis
*B. Eye-viral conjunctivitis, corneal ulcer, fulminating iritis, or panophthalmitis
C. Lacrimal passages-dacryocystitis
D. Lids-styes, vaccinia, acute meibomitis, insect bites, or vaccinal pocks
E. Nasal cavity-sinusitis
*F. Orbit-cellulitis, periostitis, dacryoadenitis, tenonitis
8. Myxedema-infiltration with mucopolysaccharides
9. Reduced plasma protein level-nephrotic state
*11. Vasomotor instability-angioneurotic edema or premenstrual phase of water retention
12. Venous congestion-local obstruction of orbital apex, carotid-cavernous fistula,
thrombosis of cavernous sinus, or right-sided heart failure
13. Whipple disease
Disdier P. et al. Chemosis associated with Whipple's disease. Am J Ophthalmol 1991;

112:217-219.
Leahey AB, et al. Chemosis as a presenting sign of systemic lupus erythematosus. Arch
Ophthalmol 1992; 110: 609-610.
CONJUNCTIVAL XEROSIS (DRYNESS OF CONJUNCTIVA)
1. Absence of blinking
acebutolol (?) ketoprofen practolol
amiodarone labetalol (?) primidone
atenolol (?) levobunolol propanolol (?)
betaxolol methyldopa propoxyphene
busulfan metoprolol (?) quinidine
chlorambucil nadolol (?) sulindac
clonidine (?) naproxen (?) thiabendazole
cyclophosphamide oxprenolol (?) timolol
doxepin perhexiline vinblastine (?)
ibuprofen pindolol (?)
3. Following cicatricial conjunctivitis (see p. 194)

4. Illness or coma
*5. Lack of closure of lids in sleep
6. Result of exposure of conjunctiva to air
A. Deficient closure of lids, such as with paralysis of orbicularis, as part of facial
palsy, spasms of the levator, or ectropion
B. Excessive proptosis, such as in exophthalmic goiter or orbital tumor
*7. Vitamin A deficiency
A. Dietary deficiencies, including malnutrition, cystic fibrosis, anorexia nervosa,
and bulimia
B. Digestive tract disorders
(1) Colitis and enteritis
*(2) In pancreas-chronic pancreatitis
(3) In stomach-achlorhydria, chronic gastritis or diarrhea, peptic ulcer
C. Hookworm disease
*D. Liver disease, such as chronic cirrhosis
E. Malaria
F. Pregnancy
G. Pulmonary tuberculosis
H. Skin disorders, such as pityriasis rubra pilaris
*I. Thyroid gland disorder, such as hyperthyroidism
J. Uyemura syndrome (fundus albipunctatus with hemeralopia and xerosis)
8. Decrease tear production
A. Congenital alacrima
B. Keratoconjunctivitis sicca
C. Riley-Day syndrome (familial dysautonomia)
D. Sjögren syndrome
E. Surgical excision of the lacrimal and accessory lacrimal glands
F. X-irradiation of the lacrimal gland
9. Following x-irradiation of the conjunctiva
Gilbert JM, et al. Ocular manifestations and impression cytology of anorexia nervosa.
Ophthalmology 1990; 97: 1001.
1991.
BITOT SPOTS
Bitot spots are small gray or white, sharply outlined areas, cheeselike or foamy,
occurring on either side of the limbus but especially in the temporal area.
1. Associated with coloboma of lid

2. Associated with corectopia, nystagmus, and absent foveal reflexes
3. Associated with Rieger anomaly
4. Congenital anomaly
5. Corneal snowflake dystrophy
6. Exposure
7. Idiopathic
*8. Keratosis follicularis (Darier -White disease) associated with retinitis pigmentosa
9. Pellagra or other poor nutritional states
10. Vitamin A deficiency
Daicker B. Ocular involvement in keratosis follicularis associated with retinitis

pigmentosa. Ophthalmologica 1995; 209:47-51.
de Keizer RJ. Conjunctival xerosis, arcus lipoides, and Rieger's disease. Doc Ophthalmol
1991; 78:365-371.
SUBCONJUNCTIVAL HEMORRHAGE (BLOOD UNDER CONJUNCTIVA)
1. Acute febrile systemic infections
A. Bacteria, such as those responsible for meningococcal septicemia, subacute

bacterial endocarditis, scarlet fever, diphtheria, typhoid fever, or cholera
B. Parasites, such as plasmodia (malaria)
C. Rickettsia, such as those causing typhus fever
D. Unknown infective agents, such as those causing glandular fever
E. Viruses, such as those responsible for influenza, smallpox, measles, yellow
fever, sandfly fever, or Kaposi sarcoma
2. Associated with use of drugs, including the following:
acetylcholine desoxycorticosterone methylprednisolone
acid bismuth sodium dexamethasone mithramycin
tartrate dibucaine mitotane
adrenal cortex injection dyclonine oxyphenbutazone
aldosterone epinephrine paramethasone
allopurinol ethambutol penicillamine
alseroxylon etidocaine phenacaine
aspirin fludrocortisone phenylbutazone
benoxinate fluorometholone phenytoin
betamethasone fluorouracil piperocaine
bismuth carbonate fluprednisolone plicamycin
bismuth oxychloride glycerin pralidoxime
bismuth salicylate heparin prednisolone
bismuth sodium tartrate hexachlorophene prednisone
bismuth sodium hydrocortisone prilocaine
thioglycollate indomethacin (?) procaine
bismuth sodium iodide and iodine proparacaine
triglycollamate solutions and propoxycaine
bismuth subcarbonate compounds (?) radioactive iodides (?)
bismuth subsalicylate isosorbide rauwolfia serpentina
bupivacaine ketoprofen rescinnamine
butacaine leuprolide acetate reserpine
chloroprocaine lincomycin sodium chloride
cobalt (?) mannitol sodium salicylate
cocaine medroxyprogesterone sulfacetamide
combination products of medrysone sulfachlorpyridazine
estrogens and mepivacaine sulfacytine
progestogens meprednisone sulfadiazine
cortisone methaqualone sulfadimethoxine
deserpidine methylphenidate sulfamerazine
sulfameter sulfapyridine tetracaine
sulfamethazine sulfasalazine triamcinolone
sulfamethizole sulfathiazole trichloroethylene
sulfamethoxazole sulfisoxazole urea
sulfamethoxypyridazine sulindac urokinase (?)
sulfanilamide syrosingopine vitamin A
sulfaphenazole tamoxifen
3. Blood dyscrasias
A. Associated with thrombocytopenia
*(1) Anemias, especially, aplastic anemia
*(2) Drugs, including the following:
absorbed acebutolol aurothioglycanide cefonicid
acebutolol azatadine cefoperazone
acenocoumarin azathioprine ceforanide
acenocoumarol bacillus Calmette Guérin cefotaxime
acetaminophen (BCG) vaccine cefotetan
acetanilid barbital cefoxitin
acetazolamide BCNU cefsulodin
acetohexamide bendroflumethiazide ceftizoxime
acetophenazine benzathine penicillin G ceftriaxone
actinomycin C benzthiazide cefuroxime
acyclovir bishydroxycoumarin cephalexin
allobarbital bleomycin cephaloglycin
allopurinol brompheniramine cephaloridine
alprazolam busulfan cephalothin
aminopterin butabarbital cephapirin
aminosalicylic acid (?) butalbital cephradine
amithiozone butallylonal chlorambucil
amitriptyline butaperazine chloramphenicol
amobarbital butethal chlordiazepoxide
amodiaquine cactinomycin chloroethyl-cyclohexyl-
amphotericin B calcifediol nitrosourea(CCNU)
ampicillin calcitriol chloroquine
anisindione captopril chlorothiazide
antimony lithium carbamazepine chlorpheniramine
thiomalate carbenicillin chlorpromazine
antimony potassium carbimazole chlorpropamide
tartrate carbinoxamine chlorprothixene
antimony sodium carisoprodol chlortetracycline
thioglycollate carmustine chlorthalidone
antipyrine carphenazine cholecalciferol
aprobarbital cefaclor cimetidine
atenolol cefadroxil cisplatin
auranofin cefamandole clemastine
aurothioglucose cefazolin clindamycin
clofibrate doxylamine indapamide
clonazepam dromostanolone indomethacin
clorazepate droperidol interferon alpha, beta, or
cloxacillin enalapril gamma
colchicine ergocalciferol iopamidol
cyclobarbital erythromycin isocarboxazid
cyclopentobarbital ethacrynic acid isoniazid
cyclophosphamide ethopropazine ketoprofen
cycloserine ethosuximide labetalol
cyclothiazide ethotoin levodopa
cyproheptadine ethoxzolamide lincomycin
cytarabine ethyl biscoumacetate lithium carbonate
dacarbazine fenfluramine lomustine
dactinomycin fenoprofen lorazepam
dapsone flecainide loxapine
daunorubicin floxuridine maprotiline
deferoxamine fluorouracil measles virus vaccine
demeclocycline fluoxymesterone mechlorethamine
desipramine fluphenazine mefenamic acid
dexbrompheniramine flurazepam melphalan
dexchlorpheniramine furosemide mephenytoin
diazepam ganciclovir mephobarbital
diazoxide gentamicin meprobamate
dichlorphenamide glutethimide mercaptopurine
dicloxacillin glyburide mesoridazine
dicumarol gold Au 198 methacycline
diethazine gold sodium thiomalate methaqualone
dihydrotachysterol gold sodium thiosulfate metharbital
diltiazem griseofulvin methazolamide
dimercaprol guanethidine methdilazine
dimethindene halazepam methicillin
dimethyl imidazole haloperidol methimazole
carboxamide (DIC) heparin methitural
dimethyl sulfoxide heptabarbital methohexital
dimethyl sulfoxide hetacillin methotrexate
(DMSO) hexethal methotrimeprazine
diphenadione hexobarbital methsuximide
diphenhydramine hydracarbazine methyclothiazide
diphenylhydantoin hydralazine methyldopa
diphenylpyraline hydralazine methylene blue
diphtheria and tetanus hydrochlorothiazide methylphenidate
toxoids and hydroflumethiazide methylthiouracil
pertussis vaccine hydroxychloroquine methyprylon
divalproex sodium hydroxyurea metolazone
doxorubicin ibuprofen metrizamide
doxycycline imipramine metronidazole
mexiletine piperacetazine testosterone
mianserin piperazine tetracycline
minocycline pipobroman thiabendazole
mitomycin polio virus vaccine thiamylal
moxalactam polythiazide thiethylperazine
mumps virus vaccine potassium penicillin G thioguanine
(live) potassium penicillin V thiopental
nadolol potassium phenethicillin thiopropazate
nafcillin potassium thioproperazine
nalidixic acid phenoxymethyl thioridazine
naproxen prazepam thiotepa
nialamide primidone thiothixene
nifedipine probarbital tocainide
nitrazepam procaine penicillin G tolazamide
nitrofurantoin procarbazine tolazoline
nitroglycerin prochlorperazine tolbutamide
nortriptyline promazine tranylcypromine
oral contraceptives promethazine trazodone
orphenadrine propiomazine triazolam
oxacillin propylthiouracil trichlormethiazide
oxazepam protriptyline triethylenemelamine
oxyphenbutazone pyrimethamine trifluoperazine
oxytetracycline quinacrine trifluperidol
paramethadione quinethazone triflupromazine
penicillamine quinidine trimeprazine
penicillin quinine trimethadione
pentobarbital ranitidine tripelennamine
perazine rifampin triprolidine
pericyazine rubella and mumps virus uracil mustard
perphenazine vaccine (live) urethan
phenacetin secobarbital valproate sodium
phenelzine semustine valproic acid
phenformin sodium vancomycin
phenindione antimonylgluconate verapamil
pheniramine stibocaptate vidarabine
phenobarbital stibophen vinbarbital
phenoxymethyl streptomycin vinblastine
phenoxymethylpenicillin streptozocin vincristine
phenprocoumon sulfonamides vitamin A
phensuximide suramin vitamin D
phenylbutazone talbutal vitamin D2
phenytoin temazepam vitamin D3
pindolol testolactone warfarin
*(3) Leukemia
(4) Septicemias
(5) Splenic disorders, such as Banti or Gaucher disease, Felty syndrome,
and hemolytic icterus
*(6) Systemic lupus erythematosus (Kaposi-Libman-Sacks syndrome)
B. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
C. Hemochromatosis
D. Schomberg disease
E. Scurvy (avitaminosis C)
F. Secondary, such as that because of nephritic, cardiac, or hepatic disease
G. Thrombocytopenia purpura
4. Fragility of vessel walls because of systemic vascular disease
A. Age
B. Arteriosclerosis
C. Diabetes
D. Hypertension
E. Nephritis
5. Gravity inversion
6. Injury to orbital or adjacent structures, such as sinus, basal skull fracture, subarachnoid
hemorrhage
7. Local acute inflammation, including, acute pneumococcal conjunctivitis, leptospirosis
ictero-hemorrhagica, epidemic typhus, and scrub typhus
8. Local trauma, including surgical trauma
9. Remote injury associated with fractured bones and fat emboli following angiography
or open heart operation causing "splinter" subconjunctival hemorrhage
10. Spontaneous during menstruation
11. Spontaneous rupture of telangiectasis, varicosities, aneurysm, or angiomatous tumor
(see p. 201)
* 12. Sudden severe venous congestion of head, including that because of coughing,
vomiting, epileptic fit, strangulation, or an orbital tumor (neuroblastoma)
13. Without apparent cause-most common
Friberg TR, Weinreb RN. Ocular manifestations of gravity inversion. JAMA 1985; 253.

1990.
Werblin TP, Peiffer RL. Persistent hemorrhage after extracapsular surgery associated
with excessive aspirin ingestion. Am J Ophthalmol 1987; 104:426.
TUMORS OF THE CONJUNCTIVA
1. Epithelial tumors
A. Keratoacanthoma
B. Dyskeratosis
(1) Epithelial plaques-leukoplakia, hereditary benign intraepithelial
dyskeratosis
(2) Intraepithelial epithelioma (Bowen disease) (61N)
C. Metastatic uveal melanoma
*D. Papilloma-including virus types 11, 16, and 18
E. Epithelioma
F. Adenoma
(1) Papillary cystadenoma lymphomatosum (Warthin tumor)
(2) Oncocytoma (oxyphil-cell adenoma)
(3) Pleomorphic adenoma of Krause glands
2. Mesoblastic tumors
A. Inflammatory hyperplasias
(1) Granuloma
(2) Plasmoma
B. Connective tissue tumors
(1) Fibroma
(2) Lipoma
(3) Myxoma
3. The reticuloses
*A. Lymphoma
B. Lymphosarcoma
*C. Mycosis fungoides
4. Vascular tumors-angiomas
A. Polymorphous hemangioma, telangiectatic granuloma, granuloma pyogenicum
B. Lymphangioma
C. Angiosarcoma monomorphous angioma, Kaposi (hemorrhagic) sarcoma
5. Pigmented tumors
*A. Nevus
B. Malignant melanoma
C. Intraepithelial melanoma-precancerous melanosis
6. Peripheral nerve tumors
A. Neurofibroma
*(1) Neurilemmoma (neurinoma, schwannoma)
(2) Malignant schwannoma (neurogenic sarcoma; neurofibrosarcoma)
(3) Plexiform neurofibromatosis
B. Tuberous sclerosis (Bourneville disease)
*C. Intrascleral nerve loops
7. Amyloidosis
8. Metastatic renal cell carcinoma
9. Trematode-induced granulomas
10. Hypertrophic discoid lupus erythematosus
Crawford JB. et al. Combined nevi of the conjunctiva. Arch Ophthalmol 1999; 117:1121-
1127.
Grossniklaus HE. et al. Hemangiopericytoma of the conjunctiva. Ophthalmology 1986;
93:265-267.
Jay V, Font RL. Conjunctival amelanotic malignant melanoma arising in primary

acquired melanosis sine pigmento. Ophthalmology 1998; 105:191-194.
Marsh WM, et al. Localized conjunctival with amyloidosis associated with extranodal
lymphoma. Ophthalmology 1987; 94:61-64.
Odrich MG, et al. A spectrum of bilateral squamous conjunctival tumors associated with
the human papillomavirus type 16. Ophthalmology 1991; 98:628-635.
Peter J, Hidayat AA. Myxomas of the conjunctiva. Am J Ophthalmol 1986; 102:80-86.
Rathinam S, et al. An outbreak of trematode-induced granulomas of the conjunctiva.

Ophthalmology 2001; 108: 1223-1229.
Sagerman RH, Abramson DH. Tumors of the eye and ocular adnexae. New York:
Pergamon Press, 1982.
Uy HS, et al. Hypertrophic discoid lupus erythematosus of the conjunctiva. Am J

Ophthalmol 1999; 127:604-605.
Ware GT, et al. Renal cell carcinoma with involvement of iris and conjunctiva. Am J
Ophthalmol 1999; 127.4: 458-459.
Winward KE, Curtin VT. Conjunctival squamous cell carcinoma in a patient with human
immunodeficiency virus infection. Am J Ophthalmol 1989:554-556.
CONJUNCTIVAL CYSTS
1. Congenital corneoscleral cyst (rare)

2. Epibulbar dermoids with cystic form
*3. Epithelial cyst
A. Apposition of folds of conjunctival mucosa (common)
*B. Downgrowth of epithelium-chronic inflammatory conditions, such as that
following inflammation of pterygium
C. Glandular retention-involvement of Krause glands in chronic inflammatory
conditions, including trachoma and pemphigus
D. Pigmented cyst appearing after prolonged topical use of cocaine or epinephrine
4. Limbal wounds with iris prolapse
5. Lymphatic cyst
*6. Muscle inclusion cyst/complication of strabismus surgery
7. Parasitic cyst such as filarial cyst
8. Traumatic cyst (epithelial implantation)
Cibis GW, Waeltermann JM. Muscle inclusion cyst as a complication of strabismus
surgery. Am J Ophthalmol 1985; 100:740-741.
Jahnle RL, et al. Conjunctival inclusion cyst simulating malignant melanoma. Am J

Ophthalmol 1985; 100:483-484.
Soong HK, et al. Corneal astigmatism from conjunctival cysts. Am J Ophthalmol 1982;
93:118.
LIMBAL MASS
1. Allergic reaction
*A. Phlyctenules
B. Vernal limbal lesions
2. Amyloid - perilimbal
3. Associated with skin disease
*A. Acne rosacea (ocular rosacea)
B. Hereditary benign intraepithelial dyskeratosis
*C. Hodgkin disease
D. Limbal squamous carcinoma in xeroderma pigmentosa
E. Pityriasis rubra pilaris
F. Psoriasis (psoriasis vulgaris)
*G. Reticulum cell sarcoma-raised, pink, smooth lesions
4. Benign nodular fascitis
*5. Dermoids
6. Ectopic lacrimal gland tissue
7. Epithelial hyperplasia
8. Fibrous histiocytoma
9. Fibroxanthoma
10. Granular cell tumor
11. Granulomas
12. Hemangioma
13. Intraepithelial epitheliomas (Bowen disease)
14. Lymphomas
15. Malignant melanomas
16. Mononucleosis (infectious)
*17. Nevi
18. Papillomas
19. Pterygia
20. Sarcomas
21. Salmon patch associated with relapsing polychondritis
22. Squamous cell carcinoma
23. Subconjunctival nodules associated with Crohn disease
24. Synthetic fiber granuloma
Charles NC, et al. Epibulbar granular cell tumor. Ophthalmology 1997; 104:1454-1456.
Engelbrecht NE, et al. Combined intraepithelial squamous neoplasia and atypical
fibroxanthoma of the cornea and limbus. Am J Ophthalmol 2000; 129:94-95.
Ferry AP. Synthetic fiber granuloma 'teddy bear' granuloma of the conjunctiva. Arch
Ophthalmol 1994; 112: 1339-1341.
Grewal RK, et al. Subconjunctival nodules: an unusual ocular complication of Crohn's

disease. Can J Ophthalmol 1994; 29:238-239.
Urback SF. Infectious mononucleosis presenting as a unilateral conjunctival tumor. Acta

Ophthalmol Scand 1993; 72: 133-135.
LARGE, FLAT, FLESHY LESIONS OF PALPEBRAL CONJUNCTIVA
1. Accidental or surgical injuries

2. Carthy disease (pyorhinoblepharostomatitis vegetans)
*3. Chalazion
*4. Embryonal rhabdomyosarcoma of children
*5. Granuloma pyogenicum
6. Ligneous conjunctivitis
7. Lymphogranuloma venereum
8. Meibomian cell carcinoma
9. Myopic infection
10. Papillary hyperplasia of vernal conjunctivitis
* 11. Syphilis
12. Tuberculosis
13. Tularemia
Friedman AH, Henkind P. Granuloma pyogenicum of the palpebral conjunctiva. Am J

Ophthalmol 1971; 71:868-872.
Pau H. Differential diagnosis of eye diseases. 2nd ed. New York: Thieme Medical, 1988.
CHRONIC OR RECURRENT ULCERS OF THE CONJUNCTIVA

1. Behçet disease
2. Crohn disease
allopurinol ferrous succinate iron sorbitex
amphotericin B ferrous sulfate phenytoin
aspirin floxuridine polysaccharide- iron
ferrocholinate fluorouracil complex
ferrous fumarate gentamicin sodium salicylate
ferrous gluconate iron dextran
4. Fungi
*5. Herpes simplex
6. Mucous membrane pemphigoid
7. Pseudomonas ulcer in patients with AIDS
8. Soft chancre
*9. Syphilis (acquired lues)
10. Tuberculosis
11. Wegener granulomatosis
2000.
Hegab SM, et al. Conjunctival ulcer in patients with Crohn's disease. Ophthalmic Surg
Lasers 1994; 25:638-639.
Jordan DR, et al. Wegener's granulomatosis: eyelid and conjunctival manifestations as

the presenting feature in two individuals. Ophthalmology 1994; 101:602-607.
PHLYCTENULAR KERATOCONJUNCTIVITIS
This condition involves a localized conjunctival, limbal, or corneal nodule

measuring about 1 to 3 mm.
* 1. Delayed hypersensitivity to bacterial protein, particularly tuberculoprotein and

staphylococci; lymphopathia venereum and coccidioidomycosis may also be allergens
2. Malnutrition
3. Secondary infection of the conjunctiva, especially from S. aureus, pneumococcus, and
Koch-Weeks bacillus
4. Systemic infection
A. Bang disease (brucellosis)
B. Candidiasis
C. Neurodermatitis
D. Mikulicz-Radecki syndrome (dacryosialoadenopathy)
E. Trachoma
F. Sjögren syndrome (secretoinhibitor syndrome)
Davis PL, Watson JL. Experimental conjunctival phlyctenulosis. Can J Ophthalmol

1969; 4:183-190.
1991.
PIGMENTATION OF THE CONJUNCTIVA (SEE PIGMENT SPOTS OF

SCLERA AND EPISCLERA)
1. Blood pigment
*A. After subconjunctival hemorrhage-red or later fine brown spots
B. Yellow tinge of malaria, blackwater fever, or yellow fever
C. Pigmentary limbal ring associated with senile, traumatic, or diseased
conditions
2. Bile pigments (yellow)-obstructive or hemorrhagic jaundice
3. Melanin pigmentation
A. Acanthosis nigricans
*B. Addison disease (adrenal cortical insufficiency)
C. Alcaptonuric ochronosis
D. Chlorpromazine (Thorazine)
E. Endogenous ochronosis
F. Keratomalacia
G. Trachoma
*H. Use of epinephrine or epinephrine bitartrate, borate, and hydrochloride
I. Vernal conjunctivitis
J. Vitiligo (leukoderma)-increased conjunctival pigmentation
K. Xeroderma pigmentosum
acid bismuth sodium bismuth subcarbonate iron dextran
tartrate bismuth subsalicylate iron sorbitol
Alcian blue captopril ketoprofen
amiodarone chloroquine methacycline
amodiaquine chlortetracycline methylene blue
amphotericin B chrysarobin minocycline
antimony lithium clofazimine minoxidil
thiomalate colloidal silver oxytetracycline
antimony potassium demeclocycline penicillamine
tartrate diethazine polysaccharide iron
antimony sodium doxycycline complex
tartrate enalapril quinacrine
antimony sodium ethopropazine rifabutin
thioglycollate ferrocholinate rifampin silver nitrate
antipyrine ferrous fumarate silver protein
bismuth carbonate(?) ferrous gluconate rose bengal
bismuth oxychloride(?) ferrous succinate sodium
bismuth salicylate(?) ferrous sulfate antimonylgluconate
bismuth sodium tartrate fluorescein stibocaptate
bismuth sodium gold AU 198 stibophen
thioglycollate (?) gold sodium thiomalate tetracycline
bismuth sodium gold sodium thiosulfate trypan blue
triglycollamate(?) hydroxychloroquine vitamin A
5. Foreign substances such as silver (argyrosis), iron (siderosis), copper (chalcosis),

arsenic (arsenic melanosis), gold (chrysiasis), aluminum, quinones, aniline dyes, and eye
cosmetics containing carbon black
*6. Benign melanosis-overactivity of melanocytes
A. Epithelial-congenital or acquired, for example., following radiation or use of
chemicals (arsenic); in Addison disease; because of chronic conjunctivitis
(trachoma, vernal conjunctivitis, onchocerciasis, keratomalacia)
B. Subepithelial-congenital or in association with melanosis oculi or nevus of Ota
7. Neoplasms
*A. Nevus-most common in children, localized stationary, elevated, cystic, mayor
may not have pigmentation
*B. Malignant melanoma arising from preexisting nevus, apparently normal
conjunctiva, or from an area of acquired pigmentation (intraepithelial melanoma);
occurs primarily in middle age; diffuse, flat, pigmentation; progressive; no cysts
C. Secondary melanotic tumors
D. Incidentally pigmented tumors, such as a melanocarcinoma
E. Secondary metastatic tumors from lung or breast
8. Ocular causes, including the following:
A. Apocrine adenocarcinomas
B. Foreign bodies
C. Hematic cysts
D. Moll gland cystadenomas
E. Staphylomas
F. Subconjunctival hematomas
Cheskes J, et al. Ocular manifestations of alkaptonuric ochronosis. Arch Ophthalmol

2000; 118:724-727.
Linebarger EJ, et al. Conjunctival aluminum deposition following pneumatic cryopexy.

Arch Ophthalmol 1999; 117:692.
Seregard S. Conjunctival melanoma. Surv Ophthalmol 1998; 42:32l-350.
DISCOLORATION OF CONJUNCTIVA
1. Red
A. Subconjunctival hemorrhage
2. Yellow
A. Bilirubinemia-obstructive or hemorrhagic jaundice
B. Picric acid
C. Leptospirosis
D. Brucellosis (Barg disease or Mediterranean fever)
E. Aromatic nitro and amino compounds
F. Conjunctival fat-occurs primarily in older and black patients
G. Blood pigment tinge of malaria, blackwater fever, and yellow fever
3. Gray (black)
*A. Argyrosis (silver)
atabrine
nitrochlorobenzene
phenols, specifically phenylic acid and carbon disulfide
C. Chrysiasis (gold)-grayish green effect
D. Arsenicals-ash white
E. Mascara
4. Brown
A. Subconjunctival hemorrhage-fine brown spots
B. Pigmentary limbal ring associated with senile, traumatic, or diseased
conditions
*C. Benign melanosis-overactivity of melanocytes
(1) Epithelial-congenital or acquired, following radiation or use of
chemicals (arsenic); in Addison disease (adrenal cortical insufficiency);
because of chronic conjunctivitis (trachoma, vernal conjunctivitis,
onchocerciasis, keratomalacia)
(2) Subepithelial-congenital or in association with melanosis oculi or
nevus of
D. Neoplasms
*(1) Nevus-most common in children, localized, stationary, elevated,
cystic, mayor may not have pigmentation
*(2) Malignant melanoma arising from preexisting nevus, apparently
normal conjunctiva, or from an area of acquired pigmentation
(intraepithelial melanoma); occurs primarily in middle age; diffuse, flat,
pigmentation; progressive; no cysts
E. Drugs, including the following:
aminoquinoline combinations chromic acid and chromates phenol
(benzoquinone, paraquinone, derivatives
hydroquinone) phenothiazine
aniline dyes sympathomimetics (adrenalin)
bromides
F. Metabolic or vitamin disturbance, including alkaptonuria

5. Blue pigmentation
A. Ink tattoo from pens
B. Manganese dust
Crawford JB, et al. Combined nevi of the conjunctiva. Arch Ophthalmol 1999; 117:1121-
1127.
SYMBLEPHARON
Symblepharon involves fusing of the eyelid to an opposing surface, such as the
tarsal and bulbar conjunctiva.
1. Physical trauma with denuded epithelium, including purulent, membranous, bullous, or

ulcerative conjunctivitis and trauma
*2. Chemical bums-especially lime or caustic bums
*3. Inflammation-especially from drug reactions, including:
allobarbital gold Au 198 silver protein
amobarbital gold sodium thiomalate sulfacetamide
aprobarbital gold sodium thiosulfate sulfachlorpyridazine
auranofin heptabarbital sulfacytine
aurothioglucose hexethal sulfadiazine
aurothioglycanide hexobarbital sulfadimethoxine
barbital idoxuridine sulfamerazine
benzalkonium idoxuridine (IDU) sulfameter
butabarbital isoflurophate sulfamethazine
butalbital mephobarbital sulfamethizole
butallylonal metharbital sulfamethoxazole
butethal methitural sulfamethoxypyridazine
carbachol (?) methohexital sulfanilamide
clonidine (?) mild silver protein sulfaphenazole
colloidal silver mitomycin sulfapyridine
cyclobarbital oxyphenbutazone sulfasalazine
demecarium penicillamine sulfathiazole
diethylpropanediol pentobarbital sulfisoxazole
(DEP) phenobarbital talbutal (?)
dipiperidinoethane phenylbutazone thiamylal
(DPE) pilocarpine thiopental (?)
dipivefrin primidone timolol
echothiophate probarbital trifluridine
epinephrine secobarbital vidarabine
F3T silver nitrate vinbarbital (?)
4. Long-standing acute inflammation

A. Pemphigus (Cazenave disease)
B. Stevens-Johnson disease (dermatostomatitis)
5. Congenital
6. Associated with cyanoacrylate tissue adhesive
Leahey AB, Gottsch JD. Symblepharon associated with cyanoacrylate tissue adhesive.
Am J Ophthalmol 1993; 115: 46-49.
Meyer SJ, et al. Conjunctival involvement in paraneoplastic pemphigus. Am J

Ophthalmol 1992; 114:621-624.
CONJUNCTIVAL CONCRETIONS
Conjunctival concretions are small yellow spots that are most common in tarsal
conjunctiva.
1. Chronic inflammatory conditions, including atopic keratoconjunctivitis, vernal
conjunctivitis, and posttrachomatous degenerations
2. Elderly
3. Calcium deposits in patients with chronic renal failure treated with maintenance
hemodialysis
Chin GN, et al. Ultrastructural and histochemical studies of conjunctival concretions.

Arch Ophthalmol 1980; 98: 720.
Pahor D, et al. Conjunctival and corneal changes in chronic renal failure patients treated
with maintenance hemodialysis. Ophthalmologica 1995; 209:14-16.
LESIONS OF CARUNCLE
1. Apocrine hydrocystoma
*2. Basal cell carcinoma
3. Capillary hemangioma
4. Chronic inflammation
5. Dermoid
6. Ectopic lacrimal gland
7. Epithelial inclusion cyst
8. Foreign-body granuloma
9. Granular cell myeloblastoma
10. Histiocytic lymphoma
11. Lipogranuloma
12. Lymphangiectasis
13. Malignant melanoma
14. Nevus
15. Normal caruncle
*16. Oncocytoma
17. Papilloma
18. Pilar cyst
19. Plasmacytoma
20. Pyogenic granuloma
21. Reactive lymphoid hyperplasia
22. Sebaceous gland hyperplasia
*23. Sebaceous gland adenoma
24. Seborrheic keratosis
25. Squamous cell carcinoma
Rennie IG. Oncocytomas of the lacrimal caruncle. Br J Ophthalmol 1980; 64:935.
Shields CL, et al. Types and frequency of lesions of the caruncle. Am J Ophthalmol 1986;
102:771-778.
Shields CL, Shields JA. Tumors of the caruncle. Int Ophthalmol

Clin 1993; 33:31-36.
6
Globe
CONTENTS
Microphthalmia 220
Buphthalmos 222
Pseudoendophthalmitis 223
Endophthalmitis 223
Intraocular cartilage 225
Intraocular calcifications 225
Intraocular adipose tissue 226
Soft globe 226
Phthisis bulbi 228
Clinical anophthalmos 228
Oculodigital stimulation 229
Anterior segment ischemia 229
MICROPHTHALMIA (SMALL GLOBE)
1. Microphthalmia associated with the following:

A. Cataract-dominant inheritance
B. Coloboma-dominant and sex-linked inheritance
C. Congenital spastic diplegia-x-linked
D. Ectopic pupils-dominant inheritance
E. Glaucoma-recessive inheritance
F. Harelip and cleft palate-autosomal recessive
G. High hypermetropia-recessive inheritance
H. Malformation of hands and feet-autosomal recessive
I. Polydactyly-autosomal recessive
J. Retinitis pigmentosa and glaucoma-dominant inheritance
2. Colobomatous microphthalmia
A. X-linked
(1) Aicardi syndrome
(2) Bloch-Sulzberger syndrome (incontinentia pigmenti)
(3) Goltz syndrome (focal dermal hypoplasia)
(4) Lenz microphthalmia syndrome
B. Autosomal recessive
(1) Cohen syndrome
(2) Ellis-van Creveld syndrome
(3) Hepatic fibrosis, polycystic kidneys, colobomas, and encephalopathy
(4) Humeroradial synostosis
(5) Kartagener syndrome
(6) Laurence-Moon-Biedl syndrome
(7) Marinesco-Sjögren syndrome
(8) Meckel syndrome
(9) Micro syndrome
(10) Sjögren-Larsson syndrome
(11) Warburg syndrome
C. Autosomal dominant
(1) Basal cell nevus syndrome
(2) Congenital contractural arachnodactyly
(3) Crouzon syndrome
(4) Stickler syndrome
(5) Treacher Collins syndrome
(6) Tuberous sclerosis
(7) Zellweger syndrome
D. Chromosomal abnormalities
(1) Deletions 4p, 4r, 11q, 13q, 18q, 18r, XO
(2) Duplications 3q, 4p, 4q, 7q, 9p, 9q, l3q, 22q
(3) Ring B syndrome
(4) Triploidy
(5) Trisomy 8, 9,13,17,18, XXX, XYY
E. Unknown cause
(1) Amniogenic band syndrome (Streeter dysplasia)
(2) Cat's-eye syndrome (Schmid-Fraccaro syndrome)
(3) CHARGE (colomba, heart disease, atresia choanae, retarded growth
and retarded growth development or central nervous system anomalies,
genital hypoplasia, and ear anomalies, or deafness) syndrome
(4) Dyscraniopygophalangea (Ullrich syndrome)
(5) Facial-clefting syndromes
(6) Frontonasal dysplasia (median cleft face syndrome)
(7) Goldenhar syndrome (oculoauriculovertebral syndrome)
(8) Hemifacial microsomia syndrome
(9) Linear sebaceous nevus syndrome
(10) Rubinstein - Taybi syndrome
3. Noncolobomatous microphthalmia
A. X-linked
(1) Anderson-Warburg syndrome
(2) Forsius-Eriksson syndrome (Aland disease)
(3) Lowe syndrome (oculocerebrorenal syndrome)
B. Autosomal recessive
(1) Cerebrooculofacioskeletal syndrome
(2) Comadi syndrome
(3) Cross syndrome
(4) Diamond-Blackfan syndrome
(5) Fanconi
(6) Obesity-cerebral-ocular-skeletal anomalies syndrome
C. Autosomal dominant
(1) Blatt syndrome
(2) Gansslen syndrome
(3) Hypomelanosis of Ito syndrome
(4) Leri syndrome
(5) Myotonic dystrophy
(6) Rieger syndrome
D. Chromosomal abnormalities
(1) Duplication 10q
(2) Trisomy 21Q syndrome
(3) Chromosome deletion Xp22.l
E. Unknown cause
(1) Arachnoidal cyst
(2) Gorlin-Chaudhry-Moss syndrome
(3) Hallerman-Streiff syndrome
(4) Hutchinson-Gilford syndrome (progeria)
(5) Krause syndrome (encephaloophthalmic)
(6) Meyer-Schwickerath and Weyers syndrome
(7) Pierre Robin syndrome
(8) Retinal disinsertion syndrome
(9) Sabin-Feldman syndrome
(10) Weyers syndrome
F. Infectious etiology
(1) Congenital rubella (Gregg syndrome)
(2) Congenital spherocytic anemia
(3) Congenital toxoplasmosis
(4) Cytomegalovirus
(5) Epstein-Barr syndrome
(6) Herpes virus
(7) Mumps
(8) Varicella
G. Intoxicants
(1) Fetal alcohol effects
(2) Maternal phenylketonuria fetal effects
4. Idiopathic
5. Nanophthalmos
Eng A, et al. Linear facial skin defects associated with microphthalmia and other
malformations, with chromosome deletion Xp22.1. J Am Acad Dermatol 1994; 31:680-
682.
BUPHTHALMOS (LARGE GLOBE)

Buphthalmos usually is associated with corneal abnormalities such as opacities
and rupture of Descemet membrane; the transition from cornea to sclera is unclear, and a
thin, bluish sclera may be present.
1. Associated with anterior chamber cleavage syndrome (Reese-Ellsworth syndrome)
2. Autosomal recessive inheritance
6. Congenital rubella syndrome (Gregg syndrome)
7. Cryptophthalmia syndrome (cryptophthalmos-syndactyly)
8. Hurler syndrome
9. Krabbe syndrome
10. Lowe syndrome (oculocerebrorenal syndrome)
11. Milroy disease (chronic hereditary edema; Noone-Milroy-Meige disease)
12. Neurofibromatosis (von Recklinghausen disease)
13. Oculodentodigital dysplasia
14. Rieger syndrome (hypodontia and iris dysgenesis)
15. Sporadic occurrence
16. Sturge-Weber syndrome (encephalotrigeminal syndrome)
Williams & Wilkins. 2002.
PSEUDOENDOPHTHALMITIS (CONDITIONS THAT SIMULATE

ENDOPHTHALMITIS)
*1. Chemical reactions from irritating chemicals (irrigating solutions or medications)

introduced into the anterior chamber
2. Foreign material in the anterior chamber
3. Metastatic carcinoma
4. Retained lenticular material
5. Severe postoperative iridocyclitis
6. Toxic anterior segment syndrome (TASS)
Levine RA, Williamson DE. Metastatic carcinoma simulating a post-operative

endophthalmitis. Arch Ophthalmol 1970; 83:59-60.
Mamalis N. Inflammation. In: Charlton JF, Weinstein GW. Ophthalmic surgery

complications: prevention and management. Philadelphia: JB Lippincott. 1995:313-338.
Monson MC, et al. Toxic anterior segment inflammation following cataract surgery. J
Cataract Ref Surg 1992;18: 184-189.
ENDOPHTHALMITIS (INTRAOCULAR INFECTION)

1. Bacterial agents
A. Gram positive
(1) Bacillus subtilis, megaterium, anthracis, cereus
(2) Clostridium peifringens (B. welchii)
(3) Clostridium tetani
(4) Coryneform bacterium
(5) Diplococcus pneumoniae (Pneumococcus)
(6) Diphtheroids
(7) Listeria monocytogenes
*(8) Propionibacterium acnes
*(9) Staphylococcus aureus, albus, and epidermidis
(10) Streptococcus viridans, S. hemolytic, S. pneumoniae, and Pyogenes
salivarius
B. Gram negative
(1) Aerobacter aerogenes
(2) Enterobacter cloacae
(3) Escherichia coli
(4) Fusobacterium organisms
(5) Klebsiella pneumoniae (Friedlander bacillus)
(6) Meningococci
(7) Morganella species
(8) Mycobacterium organisms
(9) Neisserias catarrhalis
(10) Ochrobactrum anthropi
(11) Pasteurella multocida and tularensis
(12) Proteus vulgaris (B. proteus) and mirabilis
*(13) Pseudomonas aeruginosa (B. pyocyaneus)
(14) Serratia marcescens
(15) Yersinia enterocolitica or Y. pseudotuberculosis
2. Fungal agents
A. Acanthamoeba
B. Actinomyces species, including Nocardiosis
C. Aspergillus species
D. Blastomyces dermatitidis
E. Candida species
F. Cephalosporium species, hyphas
G. Coccidioides immitis
H. Crytococcus neoformans
I. Hormodendrum
J. Hyalopus bogolepofi
K. Hyalosporus
L. Mucormycosis species
M. Neurospora sitophila
N. Sporothrix schenkii
O. Sporotrichum schenkii
P. Volutella species
3. Viral agents
A. Behçet syndrome (dermatostomatoophthalmic syndrome)
B. Cytomegalovirus
C. Myxovirus (influenza)
D. Nocardia asteroides
E. Vaccinia
F. Variola
4. Nematode agents
A. Taenia solium
B. Toxocara canis and T. cati
5. Other agents
A. Mycosis fungoides
B. Exophiala jeanselmei (yeast)
Blackman DM, et al. Bacillus cereus endopthalmitis secondary to self-inflicted periocular

injections. Arch Ophthalmol 2000; 118:1585-1586.
Chen JC, Roy M. Epidemic bacillus endophthalmitis after cataract surgery II.
Ophthalmology 2000; 107: 1038-1041.
Clark WL, et al. Treatment strategies and visual acuity outcomes in chronic postoperative
propionibacterium acnes endophthalmitis. Ophthalmology 1999; 106: 1665-1670.
Hofling-Lima AL, et al. Exophiala jeanselmei causing late endophthalmitis after cataract
surgery. Am J Ophthalmol 1999; 128, 4:512-514.
Li Suhui, et al. Unilateral blastomyces dermatitidis endophthalmitis and orbital cellulitis.

Ophthalmology 1998; 105: 1466-1470.
Okada AA, et al. Endogenous bacterial endophthalmitis: report of a ten-year retrospective

study. Ophthalmology 1994; 101:832-888.
Weishaar PD, et al. Endogenous aspergillus endophthalmitis. Ophthalmology 1998;

105:57-68.
INTRAOCULAR CARTILAGE
1. Angiomatosis of the retina
2. Chromosome deletion
3. Chronic inflammation
4. Facial nevus of Jadassohn (linear sebaceous nevus syndrome)
5. Incidental findings in microphthalmic eye, microphthalmos with cyst, microphthalmic
eye from a cyclopic orbit, in eyes with coloboma of the choroid and retina or ciliary body
6. Incontinentia pigmenti (Bloch-Sulzberger disease)
7. Persistent hyperplastic primary vitreous
8. Retinal dysplasia
9. Teratoid medulloepithelioma (dictyomas)
10. Trisomy (13-Patau syndrome) (globe less than 10 mm in diameter)
Wilkes SR, et al. Ocular malformation in association with ipsilateral facial nevus of
Jadassohn. Am J Ophthalmol 1981; 92:344-352.
INTRAOCULAR CALCIFICATIONS
1. Choroidal osteoma
2. Facial nevus of Jadassohn (linear sebaceous nevus syndrome)
3. Intraocular calcifications
A. Congenital deformity
B. Gitelman syndrome
C. Recurrent iritis and keratitis
E. Trauma (perforating, nonperforating, or surgical)
4. Intraocular sarcoma
*5. Retinoblastoma
6. Retinopathy of prematurity (end stage)
7. Sites of intraocular calcification
A. Calcific emboli of retinal and ciliary arteries
B. Cyclitic membrane
C. Lens
D. Peripapillary choroid
E. Posterior pole to ora serrata in region of choroid and pigment epithelium
F. Retina
G. Vitreous
Bourcier T, et al. Sclerochoroidal calcification associated with Gitelman syndrome. Am J

Ophthalmol 1999; 128: 767-768.
Trimble SN, et al. Spontaneous decalcification of a choroidal osteoma. Ophthalmology

1988; 95:631-634.
Wolter JR. The message of a bony lens. Ophthalmic Surg Lasers 1981; 12:332.
INTRAOCULAR ADIPOSE TISSUE

1. Congenital malformations
A. Dermoid or dermolipoma extending from the cornea or limbus into the globe
B. Malformed optic nerve
C. Persistent hyperplastic vitreous (PHPV) and other related ocular
malformations, such as microphthalmia, persistent hyaloid vessels, cataract, and
abnormal differentiation of the angle of the anterior chamber
2. Embolic phenomenon secondary to crush wounds of the thorax and abdomen or
fracture of long bones of the extremities
3. Formation of fatty tissue within the marrow spaces of metaplastic bone
4. Missile passing through orbit carrying orbital fat into the eye
Font RL, et al. Intraocular adipose tissue and persistent hyperplastic primary vitreous.
Willis R, et al. Heterotropic adipose tissue and smooth muscle in the optic disc:
association with isolated colobomas. Arch Ophthalmol 1972; 86:139-146.
SOFT GLOBE (DECREASED INTRAOCULAR PRESSURE)
* 1. Fistula from intraocular source, including penetrating intraocular trauma or surgery

and ruptured wall of the globe
*2. Laser or cryotherapy ciliodestructive procedure
3. Phthisis bulbi
4. Choroidal detachment
5. Injury to the cervical sympathetic nerve
6. Serous detachment of the retina
8. Systemic disturbances
A. Cardiac edema
B. Diabetic coma
C. Extreme or rapid dehydration because of malnutrition, cholera, or diarrhea
D. Decrease in ocular blood pressure due to hypotension, ligation of the carotid
artery, carotid occlusion, or pulseless disease (Takayasu syndrome)
E. Giant cell arteritis (temporal arteritis syndrome)
F. Leprosy (Hansen disease)
G. Parkinson disease (shaking palsy)
H. Postencephalitic syndrome following severe cerebral trauma, barbiturate
poisoning, in deep anesthesia, following leukotomy, or on the paralyzed side in
cases of cerebral hemiplegia
I. Severe abdominal disturbances, such as intestinal perforation or obstruction
J. Profound anemias
K. Uremic coma
acebutolol adrenal cortex injection amobarbital
acebutolol albuterol amyl nitrite
aceclidine alcohol antazoline
acetazolamide aldosterone aprobarbital
acetylcholine allobarbital aspirin
acetyldigitoxin alseroxylon atenolol
barbital ephedrine methacholine
beclomethasone epinephrine metharbital
bendroflumethiazide ergonovine methazolamide
benzthiazide ergotamine methitural
betamethasone erythrityl tetranitrate methohexital
betaxolol ether methoxyflurane
bupivacaine ethoxzolamide methyclothiazide
butabarbital etidocaine methyldopa
butalbital fludrocortisone methylergonovine
butallylonal fluorometholone methylprednisolone
butethal fluprednisolone methysergide
carbachol flurazepam metipranolol
carisoprodol (?) furosemide metocurine iodide
chlordiazepoxide gitalin metolazone
chlorisondamine glycerin metoprolol
chloroform guanethidine morphine
chlorothiazide haloperidol nadolol
chlorthalidone hashish naphazoline
cidofovir heparin neostigmine
clofibrate (?) heptabarbital nitroglycerin
clonidine hexamethonium nitrous oxide
cortisone hexethal norepinephrine
cyclobarbital hexobarbital opium
cyclopentobarbital hydrochlorothiazide oral contraceptives
cyclothiazide hydrocortisone ouabain
demecarium hydroflumethiazide oxprenolol
deserpidine indapamide oxygen
deslanoside insulin paramethasone
desoxycorticosterone isoflurophate pargyline
dexamethasone isosorbide pentaerythritol
diacetylmorphine isosorbide dinitrate tetranitrate
diazepam labetalol pentobarbital
dichlorphenamide lanatoside C pentolinium
diethylpropanediol levobunolol phenobarbital
(DEP) lidocaine phenoxybenzamine(? )
digitoxin mannitol phenylephrine
digoxin mannitol hexanitrate physostigmine
dimethyl tubocurarine marijuana pilocarpine
iodide mecamylamine pindolol
diphenylhydantoin medrysone polythiazide
dipiperidinoethane meperidine practolol
(DPE) mephenesin prednisolone
dipivefrin mephobarbital prednisone
dronabinol meplvacaine primidone
droperidol meprednisone pro barbital
echothiophate meprobamate(?) procaine
propranolol succinylcholine trichloroethylene
protriptyline syrosingopine trifluperidol
pyrilamine talbutal trimethaphan
tripelennamine tetraethylammonium trimethidinium
quinethazone tetrahydrocannabinol trolnitrate
rauwolfia serpentina tetrahydrozoline tubocurarine
rescinnamine thiamylal urea
reserpine thiopental urokinase
secobarbital timolol vinbarbital
sodium salicylate tolazoline vitamin A
spironolactone trichlormethiazide
10. Detachment of the ciliary body, planned or inadvertent

11. Hyperosmotic agents, such as mannitol or urea
12. Iritis or iridocyclitis
13. After central retinal vein occlusion
14. Myopia-low scleral rigidity may give false low readings with Schiötz tonometer, but
normal readings with applanation intraocular pressure
15. Herpes zoster
16. Following irradiation by roentgenograms or beta rays
17. Congenital lesions, including microphthalmos, aniridia, and coloboma
18. Concussion trauma
19. Necrosis of anterior segment of the eye
20. Idiopathic, including normal variation
Maus M, Katz JL. Choroidal detachment flat anterior chamber and hypotony as
complications of YAG laser cyclocoagulation. Ophthalmology 1990; 97:69-71.
PHTHISIS BULBI (DEGENERATIVE SHRINKAGE OF EYEBALL WITH

HYPOTONY)
1. Ciliodestructive procedures such as cyclocryotherapy or laser

2. Endophthalmitis
3. Following cataract surgery, especially with rubella syndrome (German measles)
4. Panophthalmitis
5. Severe ocular injury with loss of tissue
6. Severe uveitis
7. Sympathetic ophthalmia
8. Tumor, such as retinoblastoma or malignant melanoma
Cyclin MN, et al. Ciliodestructive procedures in glaucoma: clinical signs. Ophthalmology
1991; 12:1-15.
1991.
CLINICAL ANOPHTHALMOS (APPARENT ABSENCE OF GLOBE)

1. Anencephaly
2. Gross midline facial defects (median cleft face syndrome)
3. Dyscraniopygophalangea
4. Goldenhar syndrome (oculoauriculovertebral syndrome)
6. Hallermann-Streiff syndrome (dyscephalic mandibulo-oculofacial syndrome)
8. Idiopathic
9. Klinefelter syndrome (gynecomastia-aspermatogenesis)
10. Lanzieri syndrome (craniofacial malformations)
11. Leri syndrome (carpal tunnel syndrome)
12. Meckel syndrome (dysencephalia splanchnocystica syndrome)
13. Oculovertebral dysplasia (Weyers-Thier syndrome)
14. Otocephaly
15. Trisomy 13-15
16. Sex-linked or recessive hereditary
17. Waardenburg anophthalmia syndrome (anophthalmos with limb anomalies)-recessive
Graham CA, et al. X-linked clinical anophthalmos: localization of the gene to Xq27-
Xq28. Ophthal Paediatrics Genetics 1991; 12:43-48.
OCULODIGITAL STIMULATION
The patient presses on the globe through the lids with the index finger or hand;
the patient has poor visual acuity.
1. Bilateral congenital cataracts

2. Combined retinal detachment and congenital cataract
4. Congenital rubella syndrome (German measles)
5. Leber amaurosis congenita or other congenital retinal degeneration (Leber tapetoretinal
dystrophy syndrome)
6. Norrie disease (fetal iritis syndrome)
7. Total corneal leukoma
Franklin AH. Norrie's disease. Am J Ophthalmol 1971; 72:947-948.

Roy FH. Ocular autostimulation. Am J Ophthalmol 1967; 63:1776-1777.
ANTERIOR SEGMENT ISCHEMIA

This condition involves hypoxia with involvement of the cornea, iris, anterior
chamber, lens, and ciliary body.
1. Damage to normal intact anterior vessels

A. Pressure
(1) Scleral buckle
(2) Suture (Jensen procedure)
B. Thermal
(1) Cryotherapy
(2) Diathermy
2. Disinsertion of normal vessels (Hummelsheim or Knapp procedure)
3. Fuchs syndrome (I) (heterochromic cyclitis syndrome)
4. Hematologic abnormality
A. Extreme leukocytosis
B. Extreme thrombocytosis
C. Hyperglobulinemia
D. Red blood cell dysfunction including sickle cell trait
(1) Hemoglobinopathy
(2) Polycythemia vera (Vaquez-Osler syndrome)
5. Vessel wall abnormality (arteriosclerosis)
A. Arteriosclerosis
B. Giant cell arteritis
Birt CM, et al. Anterior segment ischemia in giant cell arteritis. Can J Ophthalmol 1994;
29:93-94.
Saunder RA, et al. Anterior segment ischemia after strabismus surgery. Surv Ophthalmol
Lasers 1995; 38:456-466.
7
Sclera
CONTENTS
Blue sclera 231

Dilated episcleral vessels 235
Episcleritis 235
Pigment spots of sclera and episclera 237
Scleritis 237
Staphyloma 239
Episcleral and scleral tumors 240
BLUE SCLERA
Blue sclera is characterized by localized or generalized blue coloration of sclera
because of thinness and loss of water content, which allow underlying dark choroid to be
seen.
1. Associated with high urine excretion

A. Folling syndrome (phenylketonuria)
B. Hypophosphatasia (phosphoethanolaminuria)
C. Lowe syndrome (oculocerebrorenal syndrome; chondroitin-4-sulfate-uria)
2. Associated with skeletal disorders
A. Brachmann-de Lange syndrome
B. Brittle cornea syndrome (blue sclera syndrome)-recessive
C. Crouzon disease (craniofacial dysostosis)
D. Hallermann-Streiff syndrome (dyscephalia mandibulooculofacial syndrome)
*E. Marfan syndrome (dystrophia mesodermalis congenita)
F. Marshall-Smith syndrome
G. McCune-Albright syndrome (fibrosus dysplasia)
H. Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome)
I. Osteogenesis imperfecta (van der Hoeve syndrome)
J. Paget syndrome (osteitis deformans)
K. Pierre Robin syndrome (micrognathia-glossoptosis syndrome)
L. Robert syndrome
M. Silver-Russell syndrome
N. Werner syndrome (progeria of adults)
3. Chromosome disorders
A. Trisomy syndrome
B. Turner syndrome
4. Ocular
*A. Congenital glaucoma
B. Myopia
*C. Repeated surgeries
D. Scleromalacia (perforans)
*E. Staphyloma
F. Trauma
5. Miscellaneous
A. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
B. Goltz syndrome (focal dermal hypoplasia syndrome)
C. Incontinentia pigmenti (Bloch-Sulzberger syndrome)
D. Lax ligament syndrome
E. Minocycline-induced
F. Oculodermal melanocytosis (nevus of Ota)
G. Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome)
H. Relapsing polychondritis
Cameron JA, et al. Epikeratoplasty for keratoglobus associated with blue sclera.
Fraunfelder FT, Randall JA. Minocycline-induced sclera pigmentation. Ophthalmology

1997; 104:936-938.
Extracted Table Blue Sclera
DILATED EPISCLERAL VESSELS

1. Carotid-cavernous fistula
2. Cavernous sinus thrombosis (Foix syndrome)
*3. Chronic respiratory diseases
*4. Glaucoma, untreated
5. Increased viscosity of circulating blood
A. Leukemia (early)
B. Polycythemia vera (erythema, Vaquez-Osler syndrome)
6. Occlusion of orbital veins of the apex of the orbit
A. Endocrine exophthalmos of rapid development
B. Inflammatory lesions
C. Orbital thrombophlebitis
D. Tumor (rare)
8. Tricuspid Incompetence
9. Uveal neoplasm with localized engorgement
Boniuk M. The ocular manifestations of ophthalmic vein and aseptic cavernous sinus
thrombosis. Trans Am Acad Ophthalmol Otolaryngol 1972; 76:1519-l534.
Minas TF, Podos SM. Familial glaucoma associated with elevated episcleral venous
pressure. Arch Ophthalmol 1968; 80:202-213.
EPISCLERITIS
Episcleritis is a benign, self-limited, nodular or diffuse disease that usually
resolves spontaneously within weeks but has a tendency to recur. Inflammation of
episcleral tissues causes discomfort rather than pain; it does not affect visual acuity. Even
recurrent attacks do not produce scleritis. Complications are minimal and include areas of
scleral transparency and localized keratitis)
*1. Idiopathic (single, short episode that does not recur)

2. Associated with the following diseases (recurrent attacks)
A. Addison syndrome (adrenal cortical insufficiency)
B. Arthritides
(1) Involving small and medium-sized vessels
a. Necrotizing granulomatous arthritis; Wegener granulomatosis
(Wegener syndrome)
b. Polyarteritis nodosa (Kussmaul disease)
(2) Involving small, medium, and large vessels
a. Arteritis in collagen vascular diseases
(i) Progressive systemic sclerosis (PSS; scleroderma)
(ii) Rheumatoid arthritis
(iii) Rheumatic fever
C. Cogan syndrome
D. Crohn disease (granulomatous ileocolitis)
E. Goodpasture syndrome (pulmonary hemosiderosis)
F. Heerfordt disease (uveoparotid fever)
G. Inflammatory pseudotumor
H. Initial manifestation of uveal melanoma (ciliary body)
I. Myeloproliferative diseases
(1) Hodgkin disease
(2) T-cell leukemia
J. Paraneoplastic syndromes
(1) Dermatomyositis
(2) Sweet syndrome (cutaneous paraneoplastic syndrome)
K. Paraproteinemia
(1) Familial Mediterranean fever
(2) Necrobiotic xanthogranuloma [increased immunoglobulin G
(IgG)/IgA]
L. Parry-Romberg syndrome (progressive hemifacial atrophy)
M. Relapsing polychondritis
N. Skin diseases
(1) Chronic cutaneous lupus erythematosus (CCLE)
(2) Erythema elevatum diutinum
(3) Lichen planus
(4) PSS; scleroderma
(5) Psoriasis
(6) Reiter syndrome (polyarteritis enterica)
(7) Wiskott-Aldrich syndrome
O. Terrien marginal corneal disease
P. Ulcerative colitis (regional enteritis)
Q. Weber-Christian disease (systemic panniculitis)
R. Pseudoepiscleritis (lesions resembling episcleritis)
(1) Conjunctivitis
(2) In-growing lash
*(3) Inflamed pinguecula
(4) Punctate keratitis
(5) Sclerosing keratitis
(6) Wegener granulomatosis
3. Drugs
A. Pamidronate disodium
4. Infectious
A. Brucellosis (Bang disease, undulant fever)
B. Coccidioidomycosis
C. Influenza
D. Leprosy (Hansen disease)
E. Leptospirosis (Weil disease)
F. Lyme disease (borreliosis, relapsing fever)
G. Lymphogranuloma venereum (Nichols-Favre disease)
H. Nematode (Angiostrongylus cantonensis)
I. Q fever
5. Trauma
*A. Episcleral foreign body
B. Following transscleral fixation of posterior chamber IOL (intraocular lens)
C. Insect bite granuloma
D. Malpositioned (Jones) tube
Akpek EK, et al. Severity of episcleritis and systemic disease association. Ophthalmology
1999; 106:729-731.
Marcarol V, Fraunfelder FT. Pamidronate disodium and possible ocular adverse drug
reactions. Am J Ophthalmol 1994; 118:220-224.
Read RW, et al. Episcleritis in childhood. Ophthalmology 1999; 106:2377-2379.
PIGMENT SPOTS OF SCLERA AND EPISCLERA

*1. Acquired melanosis
2. Cysts
acetophenazine iron sorbitex propiomazine
butaperazine mesoridazine thiethylperazine
carphenazine methdilazine thiopropazate
chlorpromazine methotrimeprazine thioproperazine
diethazine perazine thioridazine
ethopropazine pericyazine trifluoperazine
ferrocholinate perphenazine triflupromazine
ferrous fumarate piperacetazine trimeprazine
ferrous gluconate polysaccharide iron vitamin D
ferrous succinate complex vitamin D2
ferrous sulfate prochlorperazine vitamin D3
fluphenazine promazine
iron dextran promethazine
4. Extension of adjacent or underlying malignant melanoma

5. Foreign body
6. Intrascleral nerve loops with uveal pigment (painful to touch)
*7. Nevus
8. Ochronosis with melanin deposition
*9. Resolving hemorrhage
10. Staphyloma
11. Transscleral migration of pigment following cryotherapy of intraocular tumor or
trauma
12. Uveal melanocytes carried by the scleral emissaria into the episclera (most often in
eyes, with dark irides in superior, inferior temporal, and nasal quadrants in descending
frequency; conjunctiva freely movable over them)
Kampik A, et al. Ocular ochronosis. Arch Ophthalmol 1980; 98:1411.
Shields JA, et al. Uveal pseudomelanoma due to post-traumatic pigmentary migration.

SCLERITIS
Scleritis involves a potentially destructive inflammatory process that may
accompany severe systemic disease. Ocular pain occasionally radiates to the temple, jaw,
or sinuses. Women are more frequently affected than men. Most cases begin with
bilateral involvement. Early perforation of sclera is possible. The anterior portion of the
eye is affected most severely. Posterior scleritis may be a diagnostic challenge.
1. Associated with systemic disease

A. Collagen diseases
*(1) Dermatomyositis (Wagner-Unverricht syndrome)
*(2) Felty syndrome
*(3) Giant cell (temporal) arteritis
(4) Juvenile rheumatoid arthritis (Still disease)
(6) PSS; scleroderma
(8) Reiter syndrome (polyarteritis enteric a)
(9) Rheumatoid arthritis
(10) Sjögren syndrome
(11) Systemic lupus erythematosus (SLE)
(12) Wegener granulomatosis (Wegener syndrome)
B. Metabolic diseases
(1) Cretinism (hypothyroidism)
(2) Gout
(3) Porphyria cutanea tarda
C. Myeloproliferative diseases
(1) Hodgkin disease (lymph node disease)
(2) Mycosis fungoides syndrome (Sézary syndrome)
2. Infectious
A. Bacterial
(1) Leprosy
(2) Lymphogranuloma venereum (Nichols-Favre disease)
(4) Tuberculosis
B. Viral infections
(1) Herpes simplex
(2) Herpes zoster
(3) Influenza
(4) Mumps
C. Fungal-aspergillosis
D. Helminth infection-acanthamoeba
E. Protozoan-toxoplasmosis
F. Infections
(1) Associated with skin disease or immunosuppressive status
(2) Spreading directly from conjunctiva, cornea, uvea, periorbital tissues,
nose, or sinuses
3. Miscellaneous
A. Cogan syndrome
B. Crohn disease (granulomatous ileocolitis)
C. Goodpasture syndrome (pulmonary hemosiderosis)
D. Erythema nodosum
E. Exogenous infection via penetration through conjunctiva
F. Hashimoto thyroiditis
G. Heerfordt disease (uveoparotid fever)
H. Necrobiotic xanthogranuloma (increased IgG/IgA)
I. Terrien marginal corneal disease
J. Uveitis
4. Drugs
A. Pamidronate disodium
*5. Trauma-following cataract or strabismus surgery
Heiligenhaus A, et al. Ultrasound biomicroscopy in scleritis. Ophthalmology 1998;

105:527-534.
Knox C, Michele MD, et al. Brief reports. Am J Opthalmol 1997; 123:5,713-714.
Maza MS, et al. Scleritis-associated uveitis. Ophthalmology 1997; 104:58-63.
Perry SR, et al. The clinical and pathologic constellation of Wegener granulomatosis of
the orbit. Ophthalmology 1997; 104:683-694.
Riono WP, et al. Scleritis. Ophthalmology 1999; 106:1328-1333.
Tuft SJ, Shah P, et al. Posterior scleritis-an unusual manifestation of Cogan's syndrome.
Br J Rheumatol 1994; 33:774-775.
STAPHYLOMA (STRETCHING AND THINNING OF THE SCLERA WITH

INCARCERATION OF UVEAL TISSUE)
1. Collagen diseases
A. Felty syndrome
B. Rheumatoid arthritis (adult)
C. Wegener syndrome (Wegener granulomatosis)
2. Following trauma
A. Beta radiation
B. Deep scleral resection for episcleral malignancies
C. Pterygium excision and mitomycin therapy
*D. Scleral buckle removal
E. Subconjunctival injection of corticosteroids
F. Ultrasound treatment for glaucoma
3. Infectious
A. Aspergillosis
B. Herpes zoster (rare)
C. Plague (bubonic plague)
D. Syphilis
E. Tuberculosis
4. Ocular cause
A. Buphthalmos associated with increased intraocular pressure
B. Comeoscleral ectasia
C. Myopia with increased anteroposterior diameter
D. Scleritis (e.g., secondary to rheumatoid arthritis)
E. Uveitis
5. Miscellaneous
A. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
B. Endarteritis
C. Epidermolysis bullosa
D. Hyperparathyroidism
E. Meckel syndrome (dysencephalia syndrome)
F. Oculodental syndrome (Peter syndrome)
G. Porphyria cutanea tarda
Dunn JP, et al. Development of scleral ulceration and calcification after pterygium
excision and mitomycine therapy. Am J Ophthalmol 1991; 112:344.
2000.
EPISCLERAL AND SCLERAL TUMORS

1. Carcinomas
2. Choroidal melanomas
*3. Epibulbar tumor
4. Fibromas
5. Hemangiomas
6. Histiocytosis
7. Lymphomas
8. Leiomyoma (transscleral)
9. Melanoblastoma (spread from choroid)
10. Retinoblastoma
Ireland KC, et al. Sinus histiocytosis presenting as bilateral epibulbar masses. Am J

Ophthalmol 1999: 127, 3:360-361.
Perry HD. Isolated episcleral neurofibroma. Ophthalmology 1982; 89:1095.

8
Cornea
CONTENTS
Crystals of the cornea 242
Anesthesia of the cornea 246
Hyperplastic corneal nerves 247
Increased visibility of corneal nerves 248
Pigmentation of the cornea 248
Corneal edema 250
Corneal hydrops 252
Microcornea 252
Megalocornea 255
Corneal opacification in infancy 258
Band-shaped keratopathy 259
Corneal keloids 260
Punctate keratitis or keratopathy 260
Morphologic classification of punctate corneal lesions 265
Sicca keratitis 266
White rings of the cornea (Coats disease) 267
Dry spots of the cornea 267
Anterior embryotoxon (Arcus) 267
Bowman membrane folds 268
Delayed corneal wound healing 270
Anterior corneal mosaic 270
Linear opacity in superficial corneal stroma 270
Superficial vertical corneal striations 271
Dendritic corneal lesions 271
Bullous keratopathy 271
Nummular keratitis 272
Deep keratitis 272
Interstitial keratitis 272
Pannus 274
Corneal opacity-diffuse 278
Corneal opacity-localized, diffuse 278
Deep corneal stromal deposits 279
Intracorneal hemorrhage 279
Central posterior stromal corneal deposits 279
Dellen 280
Phlyctenular keratoconjunctivitis 280
Corneal ring lesion 280
Corneoscleral keratitis 281
Central corneal ulcer 281
Marginal corneal ulcers 283
Descemet membrane folds 287
Descemet membrane tears 287
Descemet membrane thickening 287
Retrocorneal pigmentation 288
Low endothelial cell count 288
Snail tracks of cornea 288
Keratoconus (conical cornea) 288
Cornea plana 289
Staphyloma of cornea 290
Whorl-like corneal lesions 290
Corneal dermoids 291
Corneal problems associated with keratotic skin lesions 293
Corneal problems associated with lid excrescences 293
Corneal disease associated with lenticular problems 293
Corneal disease associated with retinal problems 296
Corneal diseases associated with deafness 300
Trigger mechanisms for recurrent herpes simplex keratitis 301
Predisposing factors in keratomycosis 301
Sclerocornea 301
Postoperative corneal melt 302
Corneal mucous plaques 304
CRYSTALS OF THE CORNEA (DEPOSITION OF CRYSTALLINE

SUBSTANCES IN THE CORNEA)
1. Bietti marginal crystalline dystrophy
2. Calcium oxalate-dieffenbachia and other plants
3. Cholesterol crystals-primary or secondary with corneal neovascularization
*4. Crystalline dystrophy of Schnyder
5. Crystalline retinopathy
6. Cystinosis syndrome (Lignac-Fanconi syndrome)
A. Benign adult
B. Congenital
7. Drugs, such as indomethacin (Indocin), chloroquine, thioridazine (Mellaril), and
clofazimine
8. Dysproteinemia
A. Cryoglobulinemia
*B. Multiple myeloma
9. Elevated bilirubin with crystalline dystrophy
10. Fine, multicolored glittering crystals following successful transplant that later
underwent graft rejection and was treated with steroids
13. Immunoglobulin G (IgG) K monoclonal gammopathy
*14. Infectious crystalline retinopathy, usually with more indolent streptococcal and
staphylococcal species
15. Post keratoplasty (Kaye dots)
16. Renal failure
17. Subconjunctival 5-fluorouracil
18. Uremia
19. Waldenström syndrome (macroglobulinemia syndrome)
Font RL, et al. Polychromatic corneal and conjunctival crystals secondary to clofazimine
therapy in a leper. Ophthalmology 1989; 96:311-315.
Ormerod LD, et al. Paraproteinemic crystalline keratopathy. Ophthalmology 1988;

95:202-212.
Rothman RF, et al. Noninfectious crystalline keratopathy after postoperative

subconjunctival 5-fluorouracil. Am J Ophthalmol 1999; 128, 2:236-237.
Vesaluoma MH, et al. In vivo confocal microscopy of a family with Schnyder crystalline
corneal dystrophy. Ophthalmology 1999; 106:944-951.
Extracted Table Crystals of the cornea (deposition of crystalline subsyance in the

cornea)
ANESTHESIA OF THE CORNEA (HYPESTHESIA OR DIMINISHED

CORNEAL SENSATION IN TRIGEMINAL DISTRIBUTION)
1. Cornea
*A. Cerebellopontine angle tumors
B. Congenital
C. Corneal dystrophy-granular, lattice, macular, and crystalline
D. Dysautonomia
*E. Infections, including herpes zoster, herpes simplex, leprosy, and malaria
F. Inflammations, including that occurring after electrocautery of Bowman
membrane, stromal edema, vascularized scars, congestive glaucoma, exposure
keratitis, radiation damage, and vitamin A deficiency
G. Trauma, including constant wearing of contact lenses and postoperatively,
including cataract extraction and within corneal transplant, following operation
for detached retina-from an encircling band or, less frequently, a circumscribed
buckle; from refractive surgery
2. Maxillary division
A. Congenital
B. Facial trauma
C. Interruption of trigeminal nerve or gasserian ganglion, including
cerebellopontine angle tumor or other space-occupying lesion in the region of the
superior orbital fissure
D. Maxillary antrum carcinoma
E. Neoplasm, foramen rotundum, sphenopterygoid fossa
F. Orbital floor fracture
G. Perineural spread of skin carcinoma
H. Surgery for trigeminal neuralgia
3. Ophthalmic division
A. Aneurysm, cavernous sinus
B. Neoplasm, cavernous sinus
C. Neoplasm, middle fossa
D. Neoplasm, orbital apex
E. Neoplasm, superior orbital fissure
4. Syndromes and diseases
A. Adie syndrome
B. Anhidrotic ectodermal dysplasia
C. Barré Lieou syndrome (posterior cervical sympathic syndrome)
D. Diabetes mellitus-youth onset, more marked with age
E. Eaton-Lambert syndrome (myasthenic syndrome)
F. Familial corneal hypesthesia
G. Foix syndrome (cavernous sinus syndrome)
H. Gradenigo syndrome (temporal syndrome)
I. Hereditary fleck dystrophy of the cornea
J. Herpes zoster
K. Hunt syndrome (herpes zoster auricularis)
L. Hydroa vacciniforme (lower cornea)
M. Multiple sclerosis
N. Oculoauriculovertebral dysplasia (Goldenhar-Gorlin syndrome)
O. Nephropathic cystinosis
P. Passow syndrome (Bremer status dysraphicus)
Q. Psoriasis (lower cornea)
R. Riley-Day syndrome (congenital familial dysautonomia)
S. Rochon-Duvigneaud syndrome (superior orbital fissure syndrome)
T. Rollet syndrome (orbital apex-sphenoidal syndrome)
U. Scholz subacute cerebral sclerosis (arylsulfatase A deficiency syndrome)
V. Temporal arteritis syndrome (cranial arteritis syndrome)
W. Tolosa-Hunt syndrome (painful ophthalmoplegia)
X. Vitamin A deficiency
5. Toxins and drugs, including oleoresin capsicum (pepper spray)
amiodarone betaxolol carisoprodol
amitriptyline bromide chloroquine
amodiaquine carbon dioxide clorazepate
atenolol (?) carbon disulfide desipramine
diazepam methyprylon stealer
gentamicin (?) metoprolol (?) timolol
glutethimide nadolol (?) trichloroethylene
hydrogen sulfide nortriptyline trifluoperazine
hydroxychloroquine paraldehyde (Stelazine)
imipramine phencyclidine vinblastine
levobunolol pindolol (?) vincristine
meprobamate propanolol
Glaser JS. Neuro-ophthalmology, 2nd ed. Philadelphia: JB Lippincott, 1989.
Katz B, et al. Corneal sensitivity in nephropathic cystinosis. Am J Ophthalmol 1987;

104:413-416.
Zollman TM, et al. Clinical effects of oleoresin capsicum (pepper spray) on the human
cornea and conjunctiva. Ophthalmology 2000; 107:2186-2189.
HYPERPLASTIC CORNEAL NERVES

This condition involves overgrowth of corneal nerves up to 20 times the normal
number. This nonspecific change may occur in association with the following conditions:
1. Deep filiform dystrophy of Maeder and Danis

*2. Herpes simplex
*3. Herpes zoster
*4. Multiple endocrine neoplasia - type II B
5. Neurofibromatosis (von Recklinghausen syndrome)
6. Neuroparalytic keratitis
7. Normal eyes at advanced age
8. Ocular pemphigus foliaceus (Cazenave disease)
9. Opaque corneal grafts
10. Phthisis bulbi
11. Posterior polymorphous dystrophy
Charlin R. Neoplasia endocrina multiple type II-B. Arch Chit Oftal 1981; 38:21-27.
Menshen JH. Corneal nerves. Surv Ophthalmol 1974; 19:1-18.
INCREASED VISIBILITY OF CORNEAL NERVES
1. "Colloidin" skin syndrome (bullous ichthyosiform erythroderma)
2. Congenital
3. Ectodermal dysplasia (Rothmund syndrome)
4. Fuchs dystrophy
5. Ichthyosis
6. Idiopathic
*7. Keratoconus
*10. Neurofibromatosis associated with pheochromocytoma and thyroid carcinoma
(Sipple syndrome)
12. Primary amyloidosis
13. Refsum syndrome (phytanic acid storage disease)
14. Siemens disease (keratosis follicularis spinulosa decalvans)
Arffa RC. Grayson's diseases of the cornea. 3rd ed. St. Louis: Mosby-Year Book, 1991.
Pau H. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical. 1988.
PIGMENTATION OF THE CORNEA

1. Melanin pigmentation
A. Epithelial melanosis
(1) Congenital
(2) Presence of limbal malignant melanoma
(3) Sequela of trachoma and other inflammations
(4) Melanocytic migration in heavily pigmented persons
B. Stromal pigmentation such as that in ochronosis
C. Endothelial melanosis
(1) Congenital
(2) Senile
(3) Degenerative, including atrophic and inflammatory conditions (such as
cornea guttata, herpes simplex, zoster keratitis, myopia, diabetes mellitus,
senile cataract, chronic glaucoma, and melanoma)
*(4) Krukenberg spindle, with or without pigmentary glaucoma, may be
present in association with diabetes mellitus
(5) Trauma-from contusions, wounds, or intraocular operations
(6) Turks line-fine vertical line in the lower portion of the cornea
2. Hematogenous pigmentation
*A. Blood staining of the cornea, most often because of total hyphema associated
with elevated intraocular pressure
B. Hemorrhage into cornea-following subconjunctival hemorrhage and
intracorneal hemorrhage from newly formed vessels, as in interstitial keratitis or
mustard gas keratitis
C. Epithelial deposit associated with spherocytic anemia
D, Hemachromatosis
3. Metallic pigmentation
A. Copper (chalcosis)
*(1) Kayser-Fleischer ring-limbal ring associated with Wilson disease
(2) Copper foreign body in cornea or intraocular region
(3) Occupational exposure or topical therapeutic use of copper-containing
substance
(4) Advanced cirrhosis of the liver, such as that associated with parasitic
infestation (schistosomiasis)
B. Silver (argyrosis)-from topical, local, or systemic use; also occupational use
C. Gold (chrysiasis)-from topical, local, or systemic use
D. Iron (siderosis)
(1) Foreign body in cornea or intraocular area
(2) Iron lines
*a. Fleischer ring-associated with keratoconus around base of the
cone
b. Hudson-Stähli line-horizontal line at the junction of the middle
and lower one third of the cornea, believed to be related to
exposure, trauma of lid closure, and chronic corneal infection
c. Stocker line-line running parallel with head of the pterygium
d. Ferry line-associated with filtering blebs, believed to result from
minute, repeated, localized trauma caused by eyelid striking the
elevated bleb
e. Circular lesion associated with congenital spherocytosis
f. Iron lines following refractive corneal surgery, such as radial
keratotomy and photorefractive keratectomy, and laser in situ
keratomileusis (hyperopic and myopic)
E. Bismuth (bismuthiasis)-from therapeutic use
F. Arsenic melanosis
4. Drugs, discoloration, including the following:
acetophenazine auranofin bismuth subsalicylate
acid bismuth sodium aurothioglucose butaperazine
tartrate aurothioglycanide calcitriol
alcohol bismuth carbonate carphenazine
amiodarone bismuth oxychloride chloroquine
amodiaquine bismuth salicylate chlorpromazine
antimony potassium bismuth sodium tartrate chlorprothixene
tartrate bismuth sodium chlortetracycline
antimony sodium thioglycollate colloidal silver
tartrate bismuth sodium diethazine
antimony sodium triglycollamate echothiophate
thioglycolate bismuth subcarbonate epinephrine
ergocalciferol methotrimeprazine sodium
ethopropazine methylene blue antimonylgluconate
ferrocholinate mild silver protein stibocaptate
ferrous fumarate perazine stibogluconate
ferrous gluconate perhexiline stibophen
ferrous succinate pericyazine tetracycline
ferrous sulfate perphenazine thiethylperazine
fluphenazine phenylmercuric nitrate thimerosal
gold Au 198 piperacetazine thiopropazate
gold sodium thiomalate polysaccharide-iron thioproperazine
gold sodium thiosulfate complex thioridazine
hydroxychloroquine prazosin thiothixene
indomethacin prochlorperazine trifluoperazine
iodide promazine triflupromazine
iodine solution promethazine trimeprazine
iron dextran propiomazine vitamin A(?)
iron sorbitex quinacrine vitamin D
meperidine(?) quinidine vitamin D2
mercuric oxide radioactive solution vitamin D3
mesoridazine silver nitrate
methdilazine silver protein
5. Other color changes

A. White discoloration-scars, fatty degeneration or infiltration, calcified areas
B. Yellow, discoloration-hyaline or colloid degeneration, and Tangier disease
(familial deficiency of high-density lipoprotein)
C. Black discoloration-coal powder, dirt, epinephrine, or ink (tattooing)
D. Yellow-brown discoloration-Kyrle disease (hyperkeratosis follicularis et
parafollicularis in cutem penetrans)
E. Grey-black discoloration-chronic phenol exposure as carbolic acid
F. Grey-white discoloration-anesthetic cornea
G. Brown discoloration-aniline (amidobenzole), including benzoquinone and
hydroquinine
Brodrick JD. Pigmentation of the cornea. Ann Ophthalmol 1979; 11:855-861.
Krueger R, et al. Corneal iron line associated with steep central islands after
photorefractive keratectomy. J Refract Surg 1997; 13:401-403.
Lazzaro DR, et al. Corneal findings in hemochromatosis. Arch Ophthalmol 1998;

116:1531-1532.
Probst LE. Ocular copper deposition associated with benign monoclonal gammopathy
and hypercupremia, keratoconus in identical twins. Cornea 1996; 15, 1:94-98.
Probst LE, et al. Pseudo-Fleischer ring after hyperopic laser in situ keratomileusis. J
Cataract Refract Surg 1999; 25:866-870.
CORNEAL EDEMA
acetophenazine erythromycin potassium penicillin V
acetylcholine ethopropazine potassium phenethicillin
alpha-chymotrypsin fluphenazine potassium
amodiaquine hydrabamine penicillin phenoxymethyl
amphotericin B hydrogen peroxide procaine penicillin G
bacitracin hydroxychloroquine prochlorperazine
benoxinate idoxuridine promazine
benzalkonium chloride idoxuridine (IDU) promethazine
benzathine penicillin G lanatoside C proparacaine
butacaine melphalan propiomazine
butaperazine mesoridazine quinacrine
carbachol methdilazine silicone
carphenazine methicillin streptomycin
chloramphenicol methotrimeprazine tetracaine
chlorhexidine neomycin tetracycline
chloroquine penicillin thiethylperazine
chlorpromazine perazine thiopropazate
chlortetracycline pericyazine thioproperazine
cocaine perphenazine thioridazine
colistin phenacaine thiotepa
deslanoside phenoxymethyl trifluoperazine
dibucaine penicillin triflupromazine
diethazine phenylephrine trifluridine
digitoxin piperacetazine trimeprazine
digoxin piperocaine urokinase
dyclonine polymyxin B vidarabine
epinephrine potassium penicillin G vinblastine
2. Endothelial decompensation
A. Noninflammatory
*(1) Acute hydrops with keratoconus
(2) Congenital
a. Anhidrotic ectodermal dysplasia
*b. Birth trauma, typically a forceps injury
c. Congenital glaucoma
d. Congenital hereditary endothelial dystrophy
e. Posterior polymorphous dystrophy
(3) Environmental cold in trigeminal nerve palsy
(4) Essential corneal edema
(5) Failed corneal graft
(6) Metabolic such as myxedema and hypercholesteremia
(7) Neuropathic conditions
(8) Postsurgical
a. Anterior segment ischemia
b. Anterior synechiae
c. Direct mechanical damage to endothelium including argon laser
iridotomy
d. Epithelial or fibrous downgrowth
e. Osmotic, such as irrigation of cornea or anterior chamber with
distilled water
f. Plasma gas sterilization
g. Stripped Descemet membrane
h. Vitreous touch
*(9) Primary degenerative-Fuchs dystrophy
(10) Traumatic
a. Anoxia of epithelium, such as from excessive wearing of contact
lens (Sattler veil)
b. Brown-McLean Syndrome
c. Chemical, such as tear gas, hydrogen peroxide and Hibiclens
d. Exposure as in exophthalmos
e. Large epithelial defect
f. Nonpenetrating including after air bag inflation injury
g. Penetrating
h. Radiation injury such as from ultraviolet, roentgenograms,
gamma rays
i. Retained foreign body-anterior chamber
j. Sympathectomy including jugular vein catheterization and Parry-
Robson syndrome
k. Trigeminal nerve palsy with cold exposure
B. Inflammatory
(1) Any severe iritis
(2) Acute graft rejection
(3) Chandler syndrome (iridocorneal endothelial syndrome)
*(4) Herpes simplex keratitis or keratouveitis
*(5) Herpes zoster keratouveitis
(6) Retinal tacks
3. Increased intraocular pressure
*A. Acute glaucoma
B. Chronic glaucoma
(1) Minimal to moderate pressure elevations in the presence of abnormal
endothelium
(2) Prolonged moderately high elevations in the presence of normal or
near-normal endothelium
4. Hypotony
Duffy RE, et al. An epidemic of corneal destruction caused by plasma gas sterilization.
Arch Ophthalmol 2000; 118:1167-1176.
Herse P, Hooker B. Corneal edema recovery dynamics in diabetes: is the alloxan induced
diabetic rabbit a useful model? Invest Ophthalmol Vis Sci 1994; 35:310-313.
Lesher MP, et al. Corneal edema, hyphema, and angle recession after air bag inflation.
Arch Ophthalmol 1993; 111: 1320-1322.
Mietz H, et al. Acute corneal necrosis after excimer laser keratectomy for hyperopia.
Ophthalmology 1999; 106: 490-497.
Reed JW, et al. Clinical and pathologic findings of aphakic peripheral corneal edema:
Brown-McLean syndrome. Cornea 1992; 11:577-583.
Wilhelmus KR. Corneal edema following argon laser iridotomy. Ophthalmic Surg Lasers
1992;23:533-537.
Zamir E, et al. Neurotrophic corneal endothelial failure complicating acute Homer

CORNEAL HYDROPS (RUPTURES OF DESCEMET MEMBRANE WITH

CORNEA INTRALAMELLAR DISSECTION AND COLLECTION OF
AQUEOUS HUMOR)
2. Forceps injury
*3. Keratoconus
4. Pellucid marginal degeneration
5. Terrien marginal degeneration
6. Trauma, blunt
Soong HK, et al. Corneal hydrops in Terrien's marginal degeneration. Ophthalmology

1986; 93:340-343.
Taglia DP, Sugar J. Case reports and small case series. Arch Opthalmol 1997; 115:274-
275.
MICROCORNEA (CORNEA WITH A HORIZONTAL DIAMETER OF LESS

THAN 10 MM)
1. Associated ocular findings
A. Aniridia and subluxated lenses
B. Autosomal-dominant cataract and myopia
C. Autosomal-dominant cataract, nystagmus, and glaucoma
D. Axenfeld syndrome (posterior embryotoxon)
E. Colobomatous microphthalmia
F. Congenital glaucoma
G. Corectopia and macular hypoplasia
H. Hyperopia
I. Meckel syndrome (dysencephalia splanchnocystica syndrome)
J. Nanophthalmos
K. Narrow-angle glaucoma
L. Sclerocornea
2. Aberfeld syndrome (congenital blepharophimosis associated with generalized
myopathy)
3. Autosomal recessive or dominant trait
5. Cataract microcornea syndrome
6. Chromosome partial deletion (long-arm) syndrome
7. Deafness retardation, arched palate syndrome
9. Gansslen syndrome (familial hemolytic icterus)
10. Hallermann-Streiff syndrome (dyscephalic mandibulooculofacial syndrome)
11. Hemifacial microsomia syndrome (Francois Haustrate syndrome)
12. Hutchinson-Gilford syndrome (progeria)
13. Laurence-Moon-Biedl syndrome (retinitis pigmentosa-polydactylyadiposogenital
syndrome)
14. Lenz microphthalmia syndrome
15. Little syndrome (nail patella syndrome)
16. Marchesani syndrome (mesodermal dysmorphodystrophy)
17. Marfan syndrome (arachnodactyly dystrophica mesodermalis congenita)
18. Meckel syndrome (dysencephalia splanchnocystica syndrome)
19. Meyer-Schwickerath-Weyers syndrome (oculodentodigital dysplasia)
20. Microcornea, glaucoma, absent frontal sinuses
21. Micro syndrome
23. Ring chromosome
24. Roberts pseudothalidomide syndrome
25. Rubella syndrome (Gregg syndrome)
27. Schwartz syndrome (glaucoma associated with retinal detachment)
28. Smith-Magenis syndrome
29. Triploidy
30. Trisomy 13 (D trisomy, Patau syndrome)
Mamdal AK. et al. Roberts pseudothalidomide syndrome. Arch Ophthalmol 2000;
118:1462-1465.
Mollica F, et al. Autosomal dominant cataract and microcornea associated with myopia in
a Sicilian family. Clin Genet 1985; 28:42-46.
Warburg M. et al. Autosomal recessive microcephaly, microcornea, congenital cataract,

mental retardation, Optic atrophy, and hypogenitalism. Arch Dis Child 1993; 147:1309-
1312.
Extracted Table Microcornea
MEGALOCORNEA (CORNEA WITH A HORIZONTAL DIAMETER OF MORE

THAN 14 MM)

2. Autosomal dominant or recessive trait
3. Congenital glaucoma (rare)
4. Craniosynostosis
5. Down syndrome
6. Facial hemiatrophy
7. Isolated
8. Marchesani syndrome (brachymorphia with spherophakia)
9. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)
10. MMR (megalocornea-mental retardation) syndrome
11. MMMM (megalocornea, macrocephaly, mental and motor retardation) syndrome
12. Mucopolysaccharidoses I-S (Scheie syndrome)
13. Neuhauser syndrome (megalocornea-mental retardation syndrome)
14. Oculocerebrorenal syndrome (Lowe syndrome)
15. Oculodental syndrome (Peter syndrome)
17. Oxycephaly (dysostosis craniofacial dyostosis)
19. Posterior embryotoxon
20. Rieger syndrome (hypodontia and iris syndrome)
22. Sex-linked recessive trait
23. Sturge-Weber syndrome (meningocutaneous syndrome)
Arffa Re. Grayson's diseases of the cornea, 3rd ed. St. Louis: Mosby-Year Book, 1991.
Frydman M, et al. Megalocornea, macrocephaly, mental and motor retardation

(MMMM). Clin Genet 1990; 38: 149-154.
Verloes A, et al. Heterogeneity versus variability in megalocornea-mental retardation

(MMR) syndromes: report of new cases and delineation of probable types. Am J Med
Genet 1993; 46:132-137.
Extracted Table Megalocornea
CORNEAL OPACIFICATION IN INFANCY (SEE CONDITIONS SIMULATING

CONGENITAL GLAUCOMA)
*1. Birth trauma, such as Descemet membrane rupture

2. Chromosomal aberrations
A. Mongolism (Down syndrome)-trisomy 21
B. Trisomy 13 (Patau syndrome)
3. Congenital malformations
A. Amyloidosis (Lubarsch-Pick syndrome)
B. Anterior chamber cleavage syndromes
(1) Axenfeld anomaly
(2) Congenital central anterior synechiae
(3) Congenital anterior staphyloma
*(4) Peter anomaly
(5) Rieger anomaly
C. Bilateral corneal dermis-like choristomas
D. Congenital glaucoma
E. De Barsy syndrome
*F. Dermoid tumors
*G. Sclerocornea
H. Xanthomas
4. Corneal dystrophy
*A. Congenital hereditary endothelial dystrophy
B. Congenital hereditary stromal dystrophy
C. Posterior polymorphous dystrophy
5. Idiopathic
6. Inborn errors of metabolism
*A. Mucopolysaccharidoses (MPS)
(1) Hurler syndrome (MPS IN)
(2) Maroteaux-Lamy syndrome (MPS VI)
(3) Morquio-Brailsford syndrome (MPS IV)
(4) Scheie syndrome (MPS IS)
B. Lowe syndrome (oculocerebrorenal syndrome)
C. von Gierke disease (glycogen disease)
D. Corneal lipoidosis-later
E. Mucolipidosis
(1) Generalized gangliosidosis (GMI-gangliosidosis I and II)
(2) ML I (lipomucopolysaccharidosis)
(3) ML III (pseudo-Hurler polydystrophy)
F. Riley-Day syndrome (congenital familial dysautonomia)
7. Inflammatory processes
A. Corneal ulceration
B. Herpes simplex and herpes zoster
C. Interstitial keratitis
D. Rubella syndrome (German measles)
E. Savin syndrome
Kolker AE, Hetherington I. Becker-Shafter's diagnosis and therapy of glaucoma. 6th ed.
St. Louis: CV Mosby, 1989.
Topilow HW, et al. Bilateral corneal dermis-like choristomas. Arch Ophthalmol 1981;
99:1387.
BAND-SHAPED KERATOPATHY
This type of corneal opacification extends horizontally over the cornea, at the
level of the Bowman membrane, in the exposed part of the palpebral aperture.
1. Anterior mosaic dystrophy, primary type

A. Episkopi (sex-linked recessive)
B. Labrador keratopathy
2. Chemical fume related as mercury vapor or calcium bichromate vapor
3. Cyclosporine as eyedrops
4. De Barsy syndrome
5. Discoid lupus erythematosus
6. Dysproteinemia
8. High levels of visible electromagnetic radiation, such as xenon arc photocoagulation
and laser causing acute severe anterior uveitis
9. Hypercalcemia
A. Excessive vitamin D as with oral intake, Boeck sarcoid with liver involvement,
acute osteoporosis, Heerfordt syndrome, and Schaumann syndrome
B. Hyperparathyroidism
C. Hypophosphatasia (phosphoethanolaminuria)
D. Idiopathic hypercalcemia
E. Milk-alkali syndrome
F. Paget syndrome (osteitis deformans)
G. Renal failure, such as that associated with Fanconi syndrome (cystinosis)
10. Ichthyosis vulgaris
*11. Local degenerative diseases, including chronic uveitis, phthisis bulbi, absolute
glaucoma, infantile polyarthritis (Still disease), rheumatoid arthritis, interstitial keratitis,
Felty syndrome, and juvenile rheumatoid arthritis
12. Long-term miotic therapy
13. Long-term steroid phosphate preparations
14. Progressive facial hemiatrophy (Parry-Romberg syndrome)
15. Rothmund syndrome (ectodermal syndrome)
16. Silicone oil in anterior chamber
17. Traumatic-chronic exposure to irritants, such as mercury fumes, calomel, calcium
bichromate vapor, and hair
18. Tuberous sclerosis (Bourneville syndrome)
19. Tumoral calcinosis
20. Viscoat usage
22. X-linked recessive ocular dystrophy
Aldave AI, et al. Congenital corneal opacification in De Barsy syndrome. Arch

Ophthalmol 2001; 119:285-288.
Beekhuis WH, et al. Silicone oil in the anterior chamber of the eye. Arch Ophthalmol
1986; 104:793.
Feist RM, et al. Transient calcific band-shaped keratopathy associated with increased
serum calcium. Am J Ophthalmol 1992; 113:459-461.
Gutt L, et al. Band keratopathy and calcific lid lesions in tumoral calcinosis. Arch
Ophthalmol 1988; 106:725-726.
Kachi S, et al. Unusual corneal deposit after the topical use of cyclosporine as eyedrops.
Am J Ophthalmol 2000; 130:667-670.
Schlotzer-Schrehardt U, et al. Corneal stromal calcification after topical steroid-

phosphate therapy. Arch Ophthalmol 1999; 117: 1414-1416.
CORNEAL KELOIDS
2. Trauma, usually with perforation of the iris
Cibis GW, et al. Corneal keloid in Lowe's syndrome. Arch Ophthalmol 1982; 100:1795.
PUNCTATE KERATITIS OR KERATOPATHY

1. Alimentary disorders
A. Mouth
*(1) Dry mouth, as in Sjögren syndrome
(2) Ulcers, such as primary herpes, ocular cicatricial pemphigoid, and
erythema multiforme
B. Lower alimentary tract
(1) Ulcerative colitis as in Sjögren disease
(2) Mild colitis, such as that due to an adenovirus
C. Stomach
(1) Indigestion as in Sjögren syndrome and acne rosacea
2. Articular diseases
A. Psoriasis arthropathica
B. Reiter disease (polyarthritis enterica)
C. Riley-Day syndrome (congenital familial dysautonomia)
D. Sjögren syndrome (secretoinhibitor syndrome)
3. Conjunctival discharge
A. Mucoid
(1) Other types of keratoconjunctivitis sicca
(2) Sjögren disease (secretoinhibitor syndrome)
B. Mucopurulent (see p. 186)
(1) Angular blepharoconjunctivitis
(2) Erythema multiforme (Stevens-Johnson syndrome)
(3) Gonococcal
(4) Inclusion conjunctivitis (acute stage)
(5) Meningococcal
(6) Reiter disease (polyarthritis enterica)
(7) Trachoma
(8) Vernal conjunctivitis
C. Serous
(1) Adenovirus
(2) Herpes simplex
(3) Herpes zoster
(4) Inclusion conjunctivitis (later)
(5) Molluscum contagiosum
(6) Trachoma (later)
(7) Warts
4. Conjunctival inflammation
A. Cicatrizing (see p. 194)
*(1) Ocular cicatricial pemphigoid
(2) Chemical bums
*(3) Erythema multiforme (Stevens-Johnson syndrome)
(4) Diphtheria
(5) Fuchs-Salzmann-Terrien syndrome (allergic reactions from drugs)
(6) Radiation bums
*(7) Sjögren keratoconjunctivitis sicca
(8) Thermal bums
(9) Trachoma
B. Diffuse catarrhal
(1) Adenovirus
(2) Bacterial conjunctivitis
(4) Onchocerciasis syndrome (river blindness)
(5) Reiter disease (polyarthritis enterica)
(6) Superior limbic keratoconjunctivitis
(7) Vaccinia
C. Follicular (see p. 192,193)
(1) Adenovirus
(2) Herpes simplex
(3) Herpes zoster
(4) Inclusion conjunctivitis
(6) Trachoma
D. Giant papillary, such as in vernal and atopic conjunctivitis, and related to
contact users, prosthesis, and exposed sutures
E. Papillary
(1) Sjögren syndrome (secretoinhibitor syndrome)
(2) Trachoma
5. Corneal conditions
A. Deep keratitis, disciform or irregular
*(1) Herpes simplex
(2) Herpes zoster and other viral diseases
(3) Corneal dystrophy (e.g., lattice)
(4) Harlequin syndrome
B. Thinned facets because of previous ulcerative or other lesions
(1) Acne rosacea (ocular rosacea)
(3) Gorlin-Chaudhry-Moss syndrome
(4) Herpes simplex
(5) Sjögren keratoconjunctivitis sicca
C. Vascularization
(3) Ocular cicatricial pemphigoid
(4) Phlyctenular disease (see p. 214)
(5) Sjögren keratoconjunctivitis sicca
(6) Trachoma
(7) Vaccinia
D. Trauma
(1) Chemical injury
(2) Contact lens related
(3) Foreign body under upper eyelid
(4) Mild, such as eye rubbing
(5) Ultraviolet photokeratopathy
E. Thygeson superficial punctate keratitis (SPK)
6. Diseases of the lids
A. Dermatitis
(1) Psoriasis
(2) Seborrheic blepharitis
B. Ectropion (see p. 78-79)
(1) Exposure keratopathy
(2) Neuroparalytic keratopathy
C. Folliculitis (see p. 94)
(1) Blepharitis due to Demodex folliculorum
(2) Seborrheic blepharitis
(3) Staphylococcal blepharitis
D. Lid retraction (see p. 62-64)
(1) Endocrine exophthalmos
*(2) Exposure keratopathy
E. Madarosis, such as that associated with leprosy (stiff immobile lids)
F. Nodules
(3) Papilloma
(4) Warts
G. Trichiasis or entropion with traumatic keratitis
H. Vesicles or ulcers
(1) Herpes simplex
(2) Herpes zoster
(3) Ocular cicatricial pemphigoid
(4) Vaccinia
I. Floppy eyelid syndrome
7. Diseases of the skin associated with punctate keratitis
A. Acne rosacea (ocular rosacea)
B. CRST (calcinosis, cutis, Raynaud phenomenon, sclerodactyly, and
telangiectasia syndrome (calcinosis)
C. Erythema multiforme (Stevens-Johnson syndrome)
D. Follicular hyperkeratosis of the palms and soles
E. Hypertrichosis
F. Ichthyosis
G. Incontinentia pigmenti
H. Leprosy (Hansen disease)
I. Melkersson-Rosenthal syndrome (Melkersson idiopathic fibroedema)
J. Ocular cicatricial pemphigoid
K. Psoriasis
8. Genitourinary diseases associated with punctate keratitis
A. Erythema multiforme (Stevens-Johnson syndrome)
B. Inclusion blennorrhea
C. Ocular cicatricial pemphigoid
D. Reiter disease (polyarthritis enterica)
9. Keratitis associated with use of drugs, including the following:
acebutolol colchicine hexachlorophene
acetophenazine cortisone hydrocortisone
acetyldigitoxin cyclopentolate idoxuridine
acyclovir cytarabine IDU
adenine arabinoside deslanoside indomethacin
adrenal cortex injection dexamethasone iodide and iodine
alcohol dexbrompheniramine solutions and
aldosterone dexchlorpheniramine compounds
allopurinol dextran isoniazid
amantadine dibucaine isotretinoin
amphotericin B diclofenac labetalol
antazoline diethazine lanatoside C
antipyrine diethylcarbamazine levobunolol
aspirin digitoxin lidocaine
atenolol digoxin medrysone
auranofin dimethindene mepivacaine
aurothioglucose dipivalyl epinephrine mesoridazine
aurothioglycanide (DPE) methdilazine
bacitracin dipivefrin methimazole
benoxinate dorzolamide methotrexate
benzalkonium dyclonine methotrimeprazine
betamethasone emedastine difumarate methoxsalen
betaxolol emetine methylprednisolone
botulinum A toxin epinephrine methylthiouracil
brimonidine tartrate ether metipranolol
brinzolamide ethopropazine metoprolol
brompheniramine etidocaine minoxidil
bupivacaine etretinate nadolol
butacaine F3T naphazoline
butaperazine firaxetine hydrochloride neomycin
capecitabine floxuridine niacin
carbimazole flumethasone ofloxacin
carphenazine fluorometholone oral contraceptives
chlorambucil fluorouracil ouabain
chloramphenicol fluphenazine oxprenolol
chlorhexidine fluprednisolone oxyphenbutazone
chloroform flurbiprofen paramethasone
chloroprocaine fluvoxamine maleate penicillamine
chlorpheniramine framycetin perazine
chlorpromazine gentamicin pericyazine
chlorprothixene gitalin perphenazine
chlortetracycline gold Au 198 phenacaine
chrysarobin gold sodium thiomalate pheniramine
ciprofloxacin gold sodium thiosulfate phenylbutazone
cocaine guanethidine phenylephrine
pilocarpine sulfacetamide thiethylperazine
pindolol sulfachlorpyridazine thimerosal
piperacetazine sulfacytine thiopropazate
piperocaine sulfadiazine thioproperazine
polymyxin B sulfadimethoxine thioridazine
prednisolone sulfamerazine thiotepa
prilocaine sulfameter thiothixene
procaine sulfamethazine timolol
prochlorperazine sulfamethizole tobramycin
promazine sulfamethoxazole trichloroethylene
promethazine sulfamethoxypyridazine trifluoperazine
proparacaine sulfanilamide trifluorothymidine
propiomazine sulfaphenazole triflupromazine
propofol sulfapyridine trimeprazine
propoxycaine sulfasalazine trimethoprim
propylthiouracil sulfathiazole trioxsalen
quinacrine sulfisoxazole tripelennamine
radioactive iodides sulindac triprolidine
rubella virus vaccine suramin tropicamide
(live) tetracaine vidarabine
smallpox vaccine tetracycline vinblastine
sodium salicylate tetrahydrozoline
10. Limbal conditions associated with punctate keratitis

A. Focal necrotic lesions
(1) Herpes simplex
(2) Phlyctenular disease
(3) Vaccinia
B. Follicles
(2) Herpes simplex
(3) Inclusion conjunctivitis
(5) Trachoma
(6) Other viral infections
C. Nodules and plaques
(1) Avitaminosis A (Bitot spots)
(2) Bowen disease (dyskeratosis)
(3) Intraepithelial melanoma
(4) Limbal vernal conjunctivitis
11. Punctate keratitis preceded by lymphadenopathy
A. Adenovirus
B. Herpes simplex
C. Herpes zoster
D. Inclusion conjunctivitis
E. Trachoma
F. Vaccinia
12. Respiratory diseases
A. Adenovirus infections
B. Myxovirus infections (influenza, Newcastle disease, mumps)
C. Recurrent herpes complicating any fever
Ferreira RC, et al. Corneal abnormalities associated with incontinentia pigmenti. Am J

Opthalmol 1997; 123:549-551.
Jones BR. Differential diagnosis of punctate keratitis. Int Ophthalmol Clin 1962; 2:591-
611.
Sachs R. Corneal complications associated with the use of crack cocaine. Ophthalmology
1993; 100:187-191.
Yang YF, et al. Epidemic hemorrhagic keratoconjunctivitis. Am J Ophthalmol 1975;

30:192-l97.
MORPHOLOGIC CLASSIFICATION OF PUNCTATE CORNEAL LESIONS

(CLASSIFICATION BY ANATOMIC LOCATION)
1. Punctate epithelial erosions-fine, very slightly depressed spots scarcely visible without
staining with fluorescein
A. Warts
B. Artificial-silk keratitis
C. Staphylococcal blepharoconjunctivitis (lower cornea)
*D. Keratoconjunctivitis sicca (interpalpebral area)
*E. Exposure keratitis (interpalpebral area)
F. Neuroparalytic keratitis (see p. 246)
G. Ocular medications (especially those with preservatives)
H. Trichiasis
I. Trauma, mild (e.g., eye rubbing)
2. Punctate epithelial keratitis-very small, whitish flecks on the surface of the epithelium
A. Fine
(1) Scattered-staphylococcal blepharitis; viral keratitis, especially
trachoma and molluscum contagiosum, sometimes inclusion
conjunctivitis, and not infrequently herpetic keratitis and rubeola and
rubella
(2) Confluent-keratitis sicca, exposure keratitis, vernal conjunctivitis,
topical steroid-induced and early viral keratitis
B. Coarse
*(1) Thygeson superficial punctate keratitis (characteristic)
(2) Herpes zoster
(3) Adenovirus infections
(4) Early herpes simplex
(5) Acne rosacea (lower cornea)
(6) Encephalitozoon hellum
C. Areolar-spots have enlarged to occupy a large area
(1) Herpes simplex
(2) Thygeson superficial punctate keratitis
(3) Herpes zoster
(4) Vaccinia
3. Filamentary keratitis or keratopathy-formation of fine epithelial filaments that are
attached at one end
*A. Keratoconjunctivitis sicca (frequent)
B. Infections, such as that due to adenovirus, herpes, vaccinia, acne rosacea,
molluscum contagiosum, rubella, rubeola, and staphylococcus
C. Trauma, such as wounds, abrasions, exposure to shortwave diathermy, and
prolonged eye patching
D. Edema of cornea, such as that due to recurrent erosions or wearing of contact
lens
E. Sarcoid with infiltration of conjunctiva and lacrimal gland
F. Heerfordt syndrome and Mikulicz syndrome
G. After irradiation-of the lacrimal gland
H. Keratoconus (see p. 288)
I. Neuropathic keratopathy (anesthesia of cornea, p. 246)
J. Conjunctival cicatrization, such as that associated with ocular cicatricial
pemphigoid, erythema multiforme, ocular psoriasis, and advanced trachoma
K. Degenerative condition of corneal epithelium, such as in advanced glaucoma
L. Superior limbic keratoconjunctivitis
M. Hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber disease)
N. Aerosol keratitis
O. Diabetes mellitus
P. Ectodermal dysplasia
Q. Following cataract or corneal transplant surgery
R. Following patching
S. Use of diphenhydramine hydrochloride (Benadryl)
T. Idiopathic
4. Punctate subepithelial keratitis-punctate epithelial keratitis may progress to combine
epithelial and subepithelial lesions followed by healing of the epithelial component,
leaving a punctate subepithelial keratitis typical of viral punctate keratitis
A. Areolar or stellate lesions-grayish white
(1) Herpes simplex (usually)
(2) Herpes zoster
(3) Vaccinia
(5) Epstein-Barr virus infection
(6) Dimmer keratitis
(7) Brucellosis
(8) Onchocerciasis
B. Fine or medium-sized lesions, typically
(1) Adenovirus, especially types 3 and 7-grayish white
C. Yellowish tinge-typical of trachoma, inclusion conjunctivitis, acne rosacea,
and marginal “catarrhal infiltrates” associated with staphylococcal blepharitis,
Neisseria conjunctivitis, and Reiter disease
5. Punctate opacifications of Bowman membrane-gray, homogeneous, thickened spots,
often with irregular edges
A. Salzmann degeneration
B. Punctate lesion of trachoma, measles, or phlyctenular disease
Jones BR. Differential diagnosis of punctate keratitis. Int Ophthalmol Clin 1962; 2:591-
611.
Diesenhouse MC, et al. Treatment of microsporidial keratoconjunctivitis with topical

fumagillin. Am J Ophthalmol 1993; 115:293-298.
Seedor JA. et al. Filamentary keratitis associated with diphenhydramine hydrochloride.

Am J Ophthalmol 1986; 101: 376-377.
SICCA KERATITIS (DRY EYE WITH SECONDARY CORNEAL CHANGES)
1. Boeck sarcoid (Schaumann syndrome)

2. Dermatitis herpetiformis
4. Herpes simplex
5. Lye bums
*6. Ocular cicatricial pemphigoid
7. Polychondritis
*8. Sjögren syndrome (secretoinhibitor syndrome)
9. Trachoma
10. Vitamin A deficiency (xerosis)
11. Stevens-Johnson syndrome
Arffa RC. Grayson's diseases of the cornea, 3rd ed. St. Louis: Mosby-Year Book, 1991.
WHITE RINGS OF THE CORNEA (COATS DISEASE)

These rings are made up of a series of tiny white dots that may coalesce at the
level of Bowman membrane or just below it.
1. Congenital
2. Trauma
*A. Foreign body, usually metal
B. Occupational-in working with limestone, there may be deposition of some of
the substance's components, especially calcium oxide, in the cornea
3. Intraocular disease
4. Iron deposition
Miller EM. Genesis of white rings of the cornea. Am J Ophthalmol 1966; 61:904.
Nevins RC, Elliott JH. White ring of the cornea. Arch Ophthalmol 1969; 82:457.
DRY SPOTS OF THE CORNEA (PRE CORNEAL TEAR FILM DRYING IN

SPOT-WISE FASHION)
The precorneal tear film is best examined by using fluorescein and cobalt-blue
filtered light. Patients may have difficulty wearing contact lenses or may have corneal
pain. Normal tear-film breakup time is greater than seconds and averages 25 to 30
seconds.
1. Associated with corneal dellen (see p. 280)

2. Chemical bums
3. Chronic bacterial or viral conjunctivitis
4. Congenital alacrima
5. Instillation of topical anesthetic
*6. Keratitis sicca
*7. Ocular cicatricial pemphigoid
8. Ocular pemphigus (chronic cicatricial conjunctivitis)
9. Sleep apnea syndrome
10. Sometimes in elderly persons without obvious pathology
11. Stevens-Johnson syndrome (erythema multiforme)
Dohlman CH. The function of the corneal epithelium in health and disease. Invest
Ophthalmol 1971; 10:376-407.
Lemp MA, Hamill JR. Factors affecting tear film break-up in normal eyes. Arch
Ophthalmol 1973; 89:103-105.
Mojon DS, et al. Eyelid, conjunctival and corneal findings in sleep apnea syndrome.
Ophthalmology 1999; 106: 1182-1185.
ANTERIOR EMBRYOTOXON (ARCUS)

In this condition, white or gray substance is deposited at level of the Descemet
membrane and Bowman membrane initially and then in the stroma with a clear limbal
interval.
*1. Age-may be present normally in a white patient older than 40 years of age or in a
black patient older than 35 years of age
2. Alagille syndrome
3. Alport syndrome (hereditary nephritis)
4. Associated with corneal disease, such as interstitial keratitis
*5. Contralateral carotid occlusive disease-when unilateral
6. Familial hypercholesterolemia (type II, familial beta-lipoproteins and type III, familial
hyper-beta and pre-beta lipoproteins [carbohydrate-induced hyperlipemia])
7. Hereditary-autosomal dominant or autosomal recessive inheritance
8. Isolated phenomenon
9. Long exposure to irritating dust or chemicals
10. Ocular anomaly association, such as blue sclera (see p. 231), megalocornea (see p.
255), or aniridia (see p. 364-365)
11. Secondary to ocular disease, such as large corneal scars, sclerokeratitis, limbal
dermoid, nevus, or epithelial cyst
12. Schnyder crystalline dystrophy
Bagla SK, Golden RL. Unilateral arcus corneae senilis and carotid occlusive disease.
JAMA 1975; 233:450.
Chavis RM, Groshong T. Corneal arcus in Alport's syndrome. Am J Ophthalmol 1973;

75:793-794.
Hingorani M, et al. Ocular abnormalities in Alagille syndrome. Ophthalmology 1999;

106:330-337.
BOWMAN MEMBRANE FOLDS

1. Bullous keratopathy
2. Idiopathic
3. Inflammation
4. Lowering of intraocular pressure, such as occurs in association with phthisis bulbi
Duke-Elder S, Leigh AG. Diseases of the outer eye. System of ophthalmology, Vol VIII,
Part 2. St. Louis: CV Mosby, 1965.
Extracted Table Anterior corneal abnormalities
DELAYED CORNEAL WOUND HEALING

Delayed corneal wound healing because of drugs, including the following:
adenine beclomethasone cortisone
adrenal cortex injection benoxinate cytarabine
aldosterone benzalkonium desoxycorticosterone
alpha chymotrypsin(?) betamethasone dexamethasone
amphotericin B butacaine dibucaine
arabinoside chymotrypsin (?) dyclonine
azathioprine cocaine F3T
bacitracin colchicine floxuridine
fludrocortisone medrysone proparacaine
fluorometholone meprednisone sulfacetamide
fluorouracil methylprednisolone sulfamethizole
fluprednisolone paramethasone sulfisoxazole
flurbiprofen penicillamine(?) tetracaine
ganciclovir phenacaine thiotepa
gentamicin phenylephrine(?) triamcinolone
hydrocortisone piperocaine trifluorothymidine
idoxuridine prednisolone trifluridine
iodine solution prednisone vidarabine
ANTERIOR CORNEAL MOSAIC

A pattern of fluorescein pooling in corneal epithelial grooves can be induced in
any normal eye by pressure on the cornea.
1. Exophthalmos as in dysthyroid eye disease with corneal compression against the

eyelids
2. Exposure to a high-pressure fire extinguisher jet
3. Pressure on the cornea, either directly on the cornea or indirectly through the lids
Bron AJ. Anterior corneal mosaic. Br J Ophthalmol 1968; 52:659.
Frazer DG, et al. Compression keratopathy. Am J Ophthalmol 1986; 102:208-210.
LINEAR OPACITY IN SUPERFICIAL CORNEAL STROMA

1. Arc-like at superior limbus
A. Poorly fit contact lens
B. Well-fitting soft contact lens with tight eyelids
2. Central
A. Amiodarone
B. Chloroquine and hydroxychloroquine
C. Microcystic epithelial dystrophy
D. Phenothiazine
Charles NC, et al. Band-shaped and whorled microcystic dystrophy of the corneal
epithelium. Ophthalmology 2000; 107:1761-1764.
Horowitz GS, et al. An unusual corneal complication of soft contact lens. Am J

Ophthalmol 1985; 100:794-797.
SUPERFICIAL VERTICAL CORNEAL STRIATIONS-EPITHELIAL
WRINKLES CAN BE ACCENTUATED WITH FLUORESCEIN
1. Corneal surgery with corneal indentation or low intraocular pressure
2. Graves disease
3. Scarred lids
4. Soft contact lens
Blue PW, Lapiana FG. Superficial vertical corneal striations: a new eye sign of Graves'
disease. Ann Ophthalmol 1980; 12:635.
Mobilia EF, et al. Corneal wrinkling induced by ultra-thin soft contact lenses. Ann
DENDRITIC CORNEAL LESIONS (AREA OF STAINING OF CORNEA IN A

BRANCHING PATTERN)
* 1. Corneal erosions, in which the epithelium may become loose
*2. Herpes simplex
*3. Herpes zoster
4. Use of soft contact lenses
*5. Acanthamoeba keratitis
6. Latanoprost
Linquist TD, et al. Clinical signs and medical therapy of early acanthamoeba keratitis.
Margulies LJ, Mannis M. Dendritic corneal lesions associated with soft contact lenses
wear. Arch Ophthalmol 1983; 101:1551-1553.
Sudesh S, et al. Corneal toxicity associated with latanoprost. Arch Ophthalmol 1999;
117:539-540.
BULLOUS KERATOPATHY (TERMINAL STAGES OF SEVERE OR

PROLONGED EPITHELIAL EDEMA SECONDARY TO ENDOTHELIAL
DAMAGE)
1. Anterior-posterior corneal incisions for myopia

2. Anterior synechiae
3. Associated with progressive facial hemiatrophy (Parry-Romberg syndrome)
4. Birth trauma (forceps injury)
5. Chronic uveitis, especially herpes simplex or herpes zoster
6. Congenital corneal dystrophy
8. Congenital hereditary endothelial dystrophy
9. Corneal hydrops (acute keratoconus)
10. Epithelial downgrowth
* 11. Following cataract surgery with or without intraocular implantation
12. Following perforating wounds, especially when the lens capsule or vitreous is
adherent to the cornea
*13. Fuchs epithelial-endothelial dystrophy
14. Immunologic reaction after keratoplasty or endothelial decompensation
15. Iridocorneal endothelial syndrome
16. Long-standing glaucoma
18. Prolonged inflammation of corneal stroma, such as in disciform or interstitial keratitis
(rare)
19. Silicone oil in anterior chamber
Deekhuis WH, et al. Silicone oil in the anterior chamber of the eye. Arch Ophthalmol
1986; 104:793.
Grayson M. Pieroni D. Progressive facial hemiatrophy with bullous and band-shaped

keratopathy. Am J Ophthalmol 1970; 70:42-44.
Yamaguchi T, et al. Bullous keratopathy after anterior posterior radial keratotomy for
myopia and myopic astigmatism. Am J Ophthalmol 1982; 93:600-606.
NUMMULAR KERATITIS (COIN-SHAPED LESIONS OF CORNEA)

1. Brucellosis
2. Dimmers nummular keratitis
4. Herpes zoster
5. Infectious mononucleosis-Epstein-Barr virus
6. Onchocerciasis (River blindness)
7. Varicella
8. Herpes simplex
DEEP KERATITIS
1. Behçet disease (dermatostomatoophthalmic syndrome)
2. Deep pustular keratitis
3. Disciform keratitis
4. Herpes zoster
5. Keratitis profunda
6. Stromal herpes
7. Vaccinia
8. Varicella
Arffa RC. Grayson's diseases of the cornea, 3rd ed. St. Louis: Mosby-Year Book. 1991.
INTERSTITIAL KERATITIS (CORNEAL STROMAL INFLAMMATION, NOT
PRIMARILY ON ANTERIOR OR POSTERIOR SURFACES OF STROMA)
1. After bums
A. Acid
B. Alkali
2. Deep punctate
A. Influenza
B. Local trauma
C. Mumps
*D. Ophthalmic zoster
*3. Luetic (syphilis)
4. Nonluetic
A. Acanthamoeba
B. rosacea (ocular rosacea)
C. Brucellosis (Bang disease)
D. Cogan I syndrome (nonsyphilitic interstitial keratitis)
E. Epstein-Barr
F. Filariasis
G. Herpes simplex
H. Hodgkin disease (lymph node disease)
I. Leishmania species
J. Measles
K. Microsporida
L. Mycosis fungoides
M. Mumps
N. Onchocerciasis
O. Recurrent fever
P. Roberts pseudothalidomide syndrome
Q. Sarcoidosis (Schaumann syndrome)
R. Sleeping sickness (von Economo syndrome)
S. Steroid therapy
T. Topical anesthetic abuse
U. Trypanosomiasis
V. Tuberculosis (scrofulous keratitis)
W. Viral as metaherpetic keratitis
X. Corneal opacification after forceps delivery
Y. Human T-Iymphotropic virus
5. Sclerosing keratitis
A. Scleritis
(1) Foci or some local process
(2) Hennebert syndrome (luetic otitic nystagmus syndrome)
(3) Sarcoidosis syndrome (Schaumann syndrome)
(5) Tuberculosis
B. Sclerocornea
C. Brawny (gelatinous) scleritis
6. With chemical poisons
A. Arsenic
B. Gold
7. With corneal ring abscess
A. Anterior segment necrosis
(1) After circular diathermy
(2) After a "string" encircling procedure for retinal detachment
(3) After multiple extraocular muscle surgery
B. Bacillus subtilis
C. Bacterium pyocyaneum
D. Pneumococci
E. Proteus species
8. With skin disease
A. Herpes zoster
B. Incontinentia pigmenti (Bloch-Sulzberger syndrome)
C. Lichen planus
D. Molluscum contagiosum
E. Palmoplantar keratosis
F. Pityriasis rubra pilaris
G. Psoriasis
Mandal AK, et al. Roberts pseudothalidomide syndrome. Arch Ophthalmol 2000; 312-
314.
Merle H, et al. A description of human T-Iymphotropic virus type I-related chronic

interstial keratitis in 20 patients. Am J Ophthalmol 2001; 131:305-308.
PANNUS (SUPERFICIAL VASCULAR INVASION CONFINED TO A SEGMENT

OF THE CORNEA OR EXTENDING AROUND THE ENTIRE LIMBUS)
* 1. Acne rosacea
*2. Allergic marginal infiltration
*3. Anoxic contact lens overwear syndrome
4. Ariboflavinosis keratopathy
*5. Contact lens usage
6. Deerfly fever (tularemia)
7. Degenerative-blind degenerative eyes; often associated with bullous keratopathy
8. Dermatitis herpetiformis (Duhring-Brocq disease)
9. Drugs including the following:
benoxinate butacaine cocaine
benzalkonium chlorhexidine dibucaine
dyclonine oxyphenbutazone tetracaine
F3T phenacaine thimerosal
ibuprofen phenylbutazone trifluridine
idoxuridine piperocaine urokinase(?)
IDU proparacaine vidarabine
iodine solution silicone
10. Fuchs corneal dystrophy (degenerative pannus)

11. Glaucoma (degenerative pannus)
12. Haemophilus influenzae
13. Histiocytosis X (Hand-Schüller-Christian syndrome)
14. Hypoparathyroidism
15. Inclusion conjunctivitis in infants and adults (micropannus) (chlamydia)
16. Keratoconjunctivitis sicca
17. Leishmaniasis
20. Lyell disease (toxic epidermal necrolysis or scalded skin syndrome)
21. Lymphopathia venereum
22. Molluscum contagiosum
23. Ocular cicatricial pemphigoid
24. Onchocerciasis (river blindness)
25. Papilloma (wart)
26. Pellagra (avitaminosis B12)
27. Pemphigus foliaceus (Cazenave disease)
28. Phlyctenular keratoconjunctivitis (see p. 280)
29. Siemens disease (keratosis follicularis spinulosa decalvans)
*30. Staphylococcal keratoconjunctivitis (micropannus)
31. Stevens-Johnson syndrome (mucocutaneous ocular syndrome)
*32. Superior limbic keratoconjunctivitis (micropannus)
33. Terrien disease (senile marginal atrophy)
34. Trachoma
35. Tuberculosis
36. Vaccinia
37. Vernal conjunctivitis (micropannus)
38. Vitamin B12 deficiency (Addison pernicious anemia syndrome)
Dixon WS, Bron AJ. Fluorescein angiographic demonstration of corneal vascularization

in contact lens wearers. Am J Ophthalmol 1973; 75:1010-1015.
Extracted Table Pannus
CORNEAL OPACITY-DIFFUSE
1. Acromesomelic dysplasia
*2. Birth trauma
3. Cockayne syndrome
*4. Congenital hereditary endothelial dystrophy
5. Congenital hereditary stromal dystrophy
6. Cystinosis
7. Fabry syndrome
8. Fetal rubella effects
9. GM gangliosidosis type 1
10. Hurler syndrome
11. Infection
12. Maroteaux-Lamy syndrome
13. Morquio syndrome
14. Mucolipidosis III
15. Mucolipidosis IV
16. Multiple sulfatase deficiency
17. MPS VII
18. Pachyonychia congenita syndrome
19. Pena-Shokeir type II syndrome [cerebrooculofacial-skeletal (COFS) syndrome]
20. Rutherford syndrome
21. Scheie syndrome
*22. Sclerocornea
23. Seip syndrome
24. Sialidosis, Goldberg type
26. 18q syndrome
CORNEAL OPACITY-LOCALIZED, CONGENITAL
1. Acromegaloid changes, cutis verticis gyrata, and corneal leukoma

2. Aniridia
3. Autosomal dominant colomba
4. Cataract microcornea syndrome
*5. Dermoid limbal, central, and ring
7. Fetal rubella effects
8. Fetal transfusion syndrome
9. Fucosidosis
10. Group 13-trisomy phenotype
11. Keratoconus posticus circumscriptus
12. Meesman syndrome
13. Peters anomaly and short stature
14. Pilay syndrome (ophthalmomandibulomelic dysplasia)
15. Radial aplasia, anterior chamber cleavage syndrome
16. Richner-Hanhart syndrome
17. Rieger syndrome
19. Waardenburg syndrome
20. Wedge-shaped stromal opacity
21. 4p syndrome
22. 11q syndrome
23. 18q syndrome
DEEP CORNEAL STROMAL DEPOSITS

*1. Cornea farinata
2. Deep filiform dystrophy
3. Deep punctiform dystrophy associated with ichthyosis
4. Fleck corneal dystrophy
5. Gold (chrysiasis)
6. Lattice corneal dystrophy
7. Macular corneal dystrophy
8. Polymorphic amyloid degeneration
Kincaid MC, et al. Ocular chrysiasis. Arch Ophthalmol 1982; 100:1791.
Mannis MJ, et al. Polymorphic amyloid degeneration of the cornea. Arch Ophthalmol
1981; 99:1217-1219.
INTRACORNEAL HEMORRHAGE
1. Associated with intraocular surgery
2. Diseases of cornea, such as corneal ulcers and chemical burns
3. Microbial keratitis
4. Migration from subconjunctival hemorrhage
5. Mooren ulceration
6. Ocular trauma
7. Spontaneous in contact lens wearers
Hurwitz BS. Spontaneous intracorneal hemorrhage caused by aphakic contact lens wear.
Ann Ophthalmol 1981; 13: 57.
Ormerod LD, Egan KM. Spontaneous hyphaema and corneal haemorrhage as

complications of microbial keratitis. Br J Ophthalmol 1988; 71:933.
Wagoner MD, et al. Intracorneal hematoma in Mooren ulceration. Am J Ophthalmol

2000; 129:251-253.
CENTRAL POSTERIOR STROMAL CORNEAL DEPOSITS

1. Bence Jones proteinuria
2. Dysproteinemia
3. Filiform corneal dystrophy
4. Immunoglobulin deposition
A. Abnormal gamma globulin
B. Benign monoclonal gammopathy
5. Multiple myeloma
Barr CC, et al. Corneal crystalline deposits associated with dysproteinemia. Arch
Ophthalmol 1980; 98:884-889.
Yassa NH, et al. Corneal immunoglobulin deposition in the posterior stroma. Arch
Ophthalmol 1987; 105:99-103.
DELLEN
Dellen is characterized by shallow corneal excavation near the limbus, usually on
the temporal side; the base of the lesion is hazy and dry.
1. Following the wearing of contact lens

2. In elderly persons-limbal vasosclerosis
3. Lagophthalmos
4. Lengthy administration of cocaine
5. Postcataract section
*6. Swelling of perilimbal tissues
A. Allergic conjunctival edema
B. Episcleritis
*C. Filtering bleb
D. Limbal tumor
*E. Postoperative advancement of rectus muscle
F. Postoperative retinal detachment
G. Pinguecula
H. Subconjunctival effusion or injection
7. With hemeralopia
Soong HK, Quigley HA. Dellen associated with filtering blebs. Arch Ophthalmol 1983;
101:385-387.
PHLYCTENULAR KERATOCONJUNCTIVITIS
This condition is characterized by a localized conjunctival, limbal, or corneal
nodule about 1 to 3 mm in size.
*1. Delayed hypersensitivity to bacterial protein, particularly tuberculoprotein and

staphylococci; lymphopathia venereum and coccidioidomycosis may also be allergens
2. Malnutrition
3. Secondary infection of the conjunctiva, especially from Staphylococcus aureus,
pneumococcus, Koch-Weeks bacillus, chlamydia, coccidioidomycosis, and gonorrhea
4. Systemic infection
A. Bang disease (Brucellosis)
B. Candidiasis
C. Neurodermatitis
D. Mikulicz-Radecki syndrome (dacryosialoadenopathy)
E. Trachoma
F. Sjögren syndrome (secretoinhibitor syndrome)
1991.
CORNEAL RING LESION

1. Acanthamoebic keratitis
2. Associated with rheumatoid arthritis-inferior
3. Associated with Sjögren syndrome (secretoinhibitor syndrome)
4. Capnocytophaga ochracea
5. Double-ring formation-allergic keratitis
6. Marginal dystrophy-degenerative chronic corneal lesion with stromal thinning and
intact epithelium
7. Marginal ulceration-secondary to massive granuloma of sclera or necrotizing nodular
scleritis (see p. 237)
8. Mooren ulcer-deeply undermined central edges and chronic course with inflammation,
painful
9. Ring abscess-rapidly destructive purulent lesion in the deepest parts of the cornea
10. Ring ulcer-see marginal corneal ulcers (p. 283-286)
11. Steroid use in furrow dystrophy
12. Terrien marginal degeneration-usually begins superiorly
13. Wegener granulomatosis (Wegener syndrome)
Daut PM, et al. Chronic exposure keratopathy complicating surgical correction of ptosis
in patients with chronic progressive external ophthalmoplegia. Am J Ophthalmol 2000;
130:519-521.
Heidemann DG, et al. Necrotizing keratitis caused by Capnocytophaga ochracea. Am J
Ophthalmol 1988; 105: 655-660.
Theodore FH, et al. The diagnostic value of a ring infiltrate in acanthamoebic keratitis.
Ophthalmology 1985; 92: 1471-1480.
CORNEOSCLERAL KERATITIS
1. Boeck sarcoid (Schaumann syndrome)
4. Infections (e.g., pseudomonas)
5. Malformations, such as in sclerocornea (see p. 301)
6. Sarcoma
8. Trisomy 13 (trisomy D)
9. Tuberculosis
CENTRAL CORNEAL ULCER

1. Bacterial origin
*A. Diplococcus pneumoniae (pneumococcus)-infiltrated gray-white or yellow
disc-shaped central ulcer typically associated with diffuse keratitis, severe
iridocyclitis, and hypopyon; follows corneal abrasion; occurs especially in
the presence of chronic dacryocystitis; enhanced by general debility
*B. Beta-hemolytic streptococcus and other streptococcus species
*C. Pseudomonas aeruginosa but may also have Pseudomonas acidovorans,
Pseudomonas stutzeri, Pseudomonas mallei, and Pseudomonas
pseudomallei-primary corneal involvement, rapid spread often to
panophthalmitis, large hypopyon, thick, greenish pus; may be contaminant of
eserine and fluorescein often is associated with contact use.
D. Escherichia coli
E. Moraxella liquefaciens (diplococcus of Petit)-morphologically resembles
diplobacillus of Morax-Axenfeld, which is never seen in central corneal
ulcers
F. Klebsiella pneumoniae
G. Proteus vulgaris
H. Actinomyces
I. Tuberculous-secondary to conjunctival or uveal infections
J. Serratia marcescens-gram-negative coccobacillus
*K. Staphylococcus aureus, S. epidermidis, and other Staphylococcus species
L. Mima polymorpha
M. Dysgonic fermenter-2
N. Others
2. Viral origin
*A. Herpes simplex virus
B. Herpes zoster
C. Vaccinia virus
D. Variola
E. Others
3. Mycotic origin-follows corneal trauma, such as foreign bodies in the cornea or corneal
abrasions caused by vegetable matter, or diseases, such as radiation keratitis, exposure
keratitis, herpes zoster, and ocular pemphigus; chronic course; shallow crater; absent
corneal vascularization; may follow treatment with antibiotics or, more likely,
treatment with steroid-antibiotic combinations
A. Absidia corymbifera
*B. Aspergillus species
C. Blastomyces dermatitidis
*D. Candida albicans
E. Cephalosporium species
*F. Fusarium solani
G. Nocardia species
H. Others
4. Acquired immune deficiency syndrome (AIDS) related
5. Atopic
6. Basement membrane abnormalities as microcysts, evidence of map, dot fingerprints, or
anterior stromal dystrophies, trauma history, other dystrophy
7. Brittle cornea syndrome
6. Chemical-latex keratitis, alkali/acid burn
*8. Dry eyes, including Sjögren syndrome
9. Exposure as lagophthalmos, lid abnormalities, inadequate blink, facial palsy, proptosis,
thyroid disease
10. Extrusion of anterior chamber intraocular lens
11. Factitious
12. Hypogammaglobulinemia
13. Medicamentosus as drops
14. Neurotrophic
15. Thermal/radiation burns
16. Sjögren syndrome
17. Soluble tyrosine aminotransferase (STAT) deficiency
Akpek EK, et al. Bilateral consecutive central corneal perforations associated with
hypogammaglobulinemia. Ophthalmology 2000; 107:123-126.
Alexandrakis G, et al. Capnocytophaga keratitits. Ophthalmology 2000; 107:1503-1506.
Kiel RJ, et al. Corneal perforation caused by dysgonic fermenter-2. JAMA 1987;
257:3269-3270.
Marshall DH, et al. Post-traumatic corneal mucormycosis caused by Absidia corymbifera.

Ophthalmology 1997; 104:1107-1111.
McKnight GT, et al. Transcorneal extrusion of anterior chamber intraocular lenses. Arch
Ophthalmol 1987; 105: 1656-1664.
Santos C, et al. Bilateral fungal corneal ulcers in a patient with AIDS-related complex.
Am J Ophthalmol 1986; 102:108-109.
MARGINAL CORNEAL ULCERS

1. Ring ulcers-often bilateral, circumcorneal injection, and continuous ring or confluent
multiple lesions
A. Acute leukemia
B. Bacillary dysentery
C. Brucellosis (Bang disease)
*D. Coalescence of several marginal ulcers
E. Dengue fever
F. Gold poisoning
G. Gonococcal arthritis
H. Following penetrating keratoplasty
I. Hookworm infestation
J. Influenza
K. Last stages of trachoma, secondary to small circumferential pannus
*L. Mooren ulcer
M. Polyarteritis nodosa (Kussmaul disease)
N. Porphyria
*O. Rheumatoid arthritis-Sjögren syndrome (secretion inhibitor syndrome)
P. Scleroderma (progressive systemic sclerosis)
Q. Systemic lupus erythematosus (Kaposi-Libman-Sacks syndrome)
R. Tuberculosis
S. Wegener granulomatosis (Wegener syndrome)
2. Simple marginal ulcers-superficial crescentic gray-colored ulcer
A. Infection-due to Staphylococcus organisms, Koch-Weeks bacillus,
pseudomonas, diplobacillus of Morax-Axenfeld; usually chronic
B. Toxic or allergic including antiinflammatory drugs
C. Systemic disturbances, such as
(1) Acute upper respiratory infection
(2) Bacillary dysentery
(3) Barre-Lieou syndrome (posterior cervical sympathetic syndrome)
(4) Brucellosis (Bang disease)
(5) Crohn disease (granulomatous ileocolitis)
(6) Gout (hyperuricemia)
(7) Influenza
(8) Lupus erythematosus (Kaposi-Libman-Sacks syndrome)
(10) Postvaccinial ocular syndrome
*(11) Rheumatoid arthritis-inferior cornea
Frueh BE, et al. Mycobacterium szulgai keratitis. Arch Ophthalmol 2000; 118:1123-
1124.
Lin JC, et al. Corneal melting associated with use of topical nonsteroidal anti-
inflammatory drugs after ocular surgery. Arch Ophthalmol 2000; 118:1129-1132.
Mondino BJ, et al. Mooren's ulcer after penetrating keratoplasty. Am J Ophthalmol 1987;
103:53-56.
Parker AV, et al. Pseudomonas corneal ulcers after artificial fingernail injuries. Am J
Ophthalmol 1989; 107:548-560.
Extracted Table Marginal corneal ulcers
DESCEMET MEMBRANE FOLDS (USUALLY FOLLOWING HYPOTONY)

*1. Trauma, such as that due to cataract or corneal surgery
2. Mechanical cause, such as firm, prolonged ocular bandaging or phthisis bulbi
3. Inflammatory condition, such as that following interstitial or herpes simplex keratitis
4. Diabetes (8%-33%)
5. Ochronosis
6. Toxic
A. Quinone and hydroquinone-vertical folds
B. Formaldehyde 26%
C. Experimental cold injury to cornea
D. Digitoxin
7. Idiopathic
Angell LK, et al. Visual prognosis in patients with ruptures in Descemet's membrane due
to forceps injuries. Arch Ophthalmol 1981; 99:2137.
DESCEMET MEMBRANE TEARS (HAAB STRIAE)

*1. Acute hydrops of the cornea, such as that due to keratoconus
*2. Buphthalmos (e.g., from congenital glaucoma)
3. Conical cornea
4. Myopia with marked anteroposterior diameter
*5. Trauma, such as birth injury or contusion
Angell LK, et al. Visual prognosis in patients with ruptures in Descemet's membrane due
to forceps injuries. Arch Ophthalmol 1981; 99:2137.
Cibis OW, Tripathi RC. The differential diagnosis of Descemet's tears (Haab's striae) and
posterior polymorphous dystrophy bands. Ophthalmology 1982; 89:614.
DESCEMET MEMBRANE THICKENING

*1. Central cornea guttata
A. Primary
B. Secondary cornea guttata
*(1) Congenital luetic interstitial keratitis
(2) Endothelial cell insult
a. Breaks in Descemet membrane, including scrolls of Descemet
membrane in healed syphilitic interstitial keratitis
b. Chandler syndrome (iridocorneal endothelial syndrome)
c. Cogan-Reese syndrome (iris-nevus syndrome)
*d. Corneal dystrophy, including Fuchs syndrome
e. Posterior keratoconus syndrome
C. Transient cornea guttata associated with short-term episodes of iritis and
corneal inflammation
2. Peripheral Hassall Henle warts
Alvarado JA, et al. Pathogenesis of Chandler's syndrome, essential iris atrophy, and the
Cogan-Reese syndrome. Invest Ophthalmol Vis Sci 1986; 27:873-882.
Rodrigues MM, et al. Fuchs' corneal dystrophy: a clinicopathologic study of the variation
in corneal edema. Ophthalmology 1986; 98:789-796.
Scattergood KD, et al. Scrolls of Descemet's membrane in healed syphilitic interstitial

keratitis. Ophthalmology 1983; 90: 1518-1523.
RETROCORNEAL PIGMENTATION
1. Endothelial phagocytosis of free melanin pigment, such as Krukenberg spindle
2. Iris melanocytes, iris pigment epithelial cells, or pigment containing macrophages in
the posterior corneal surface; can follow operative or accidental ocular trauma
3. Status post hyphema
Snip RC, et al. Posterior corneal pigmentation and fibrous proliferation by iris
melanocytes. Arch Ophthalmol 1981; 99:1232.
LOW ENDOTHELIAL CELL COUNT (DIMINISHED NUMBER OF CORNEAL

ENDOTHELIAL CELLS)
1. Acute and chronic uveitis

*2. Corneal endothelial dystrophy
3. Following cataract or other intraocular surgery
4. Cornea guttata, endothelial dystrophy, and Fuch dystrophy
Olsen T. Changes in the corneal endothelium after acute anterior uveitis as seen with the
specular microscope. Acta Ophthalmol Scand 1980; 58:250.
Murrell WJ, et al. The corneal endothelium and central corneal thickness in pigmentary
dispersion syndrome. Arch Ophthalmol 1986;104:845-846.
SNAIL TRACKS OF CORNEA

This condition involves irregular, discontinuous grayish white streaks or patches,
usually orientated horizontally and obliquely on the corneal endothelium.
1. Corneal buttons preserved in corneal storage medium

2. Following ocular surgery
3. Ocular trauma
Alfonso E, et al. Snail tracks of the corneal endothelium. Ophthalmology 1986; 99:344-
349.
KERATOCONUS (CONICAL CORNEA)

Keratoconus is characterized by noninflammatory ectasia of the cornea in its axial
part, with considerable visual impairment because of development of a high degree of
irregular myopic astigmatism. Keratoconus may be associated with
1. Acute hydrops of the cornea

3. Anetoderma and bilateral subcapsular cataracts
4. Angelman syndrome
5. Aniridia
6. Apert syndrome (acrodysplasia)
7. Asthma, hay fever
*8. Atopic dermatitis, keratosis plantaris, and palmaris
9. Autographism
10. Avellino dystrophy
11. Blue sclerotics, including van der Hoeve syndrome (osteogenesis imperfecta) (see
blue sclera, p. 231)
12. Chandler syndrome (iridocorneal endothelial syndrome)
13. Congenital hip dysplasia
14. Wearing of contact lens
15. Crouzon syndrome
16. Deep filiform corneal dystrophy
17. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata, cutis hyperelastica)
18. Essential iris atrophy
19. Facial hemiatrophy
*20. Familial
21. False chordae tendineae of left ventricle
22. Fleck corneal dystrophy
23. Focal dermal hypoplasia (Goltz syndrome)
24. Fuchs corneal endothelial dystrophy
26. Hereditary history
27. Hyperextensible joints and mitral valve prolapse
28. Hyperornithemia
29. Infantile tapetoretinal degeneration of Leber
30. Iridocorneal dysgenesis
31. Iridoschisis
32. Joint hypermobility
33. Kurz syndrome
34. Laurence-Moon-Biedl syndrome (retinitis-polydactyly-adiposogenital syndrome)
36. Lymphogranuloma venereum
38. Measles retinopathy
39. Microcornea
40. Mongolism (Down syndrome)
41. Mulvihil-Smith syndrome
42. Neurocutaneous angiomatosis
43. Neurodermatitis
46. Ocular hypertension
47. Pellucid marginal corneal degeneration
48. Posterior ectasia following laser in situ keratomileusis (generally if stromal bed less
than 250 μ)
49. Posterior lenticonus
51. Retinal disinsertion syndrome
53. Retinopathy of prematurity
54. Rieger syndrome
55. Tourette disease
56. Thalassemia syndrome
*57. Trauma, such as rubbing of eyes, birth injury, or contusion
58. Vernal catarrh
59. Vernal conjunctivitis
60. 18q syndrome
Geggel HS, Talley AR. Delayed onset keratectasia following laser in situ keratomileusis.
J Cataract Refract Surg 1999; 25:582-586.
Krachner JH, et al. Keratoconus and related noninflammatory corneal thinning disorders.
Surv Ophthalmol 1984; 28:293-322.
Lipman RM, et al. Keratoconus and Fuchs' corneal endothelial dystrophy in a patient and
her family. Arch Ophthalmol 1990; 108:993-994.
Rabinowitz YS. Keratoconus. Surv Ophthalmol 1998; 42:297-319.
Vinokur ET, et al. The association of keratoconus, hyperextensible joints, and mitral
valve prolapse. Ophthalmology 1986; 93:95.
CORNEA PLANA (DECREASED CORNEAL CURVATURE)

1. Isolated
2. Marfan syndrome
*3. Sclerocornea
STAPHYLOMA OF CORNEA (CORNEAL STRETCHING WITH

INCARCERATION OF UVEAL TISSUE)
1. Advanced keratoconus (see p. 288)
2. Avitaminosis A with keratomalacia
3. Congenital
*4. Following corneal ulcer (see p. 281, 283), neuroparalytic keratitis, corneal leprosy,
and severe corneal injury
5. Mucoviscidosis (cystic fibrosis of the pancreas)
WHORL-LIKE CORNEAL LESIONS

*1. Amiodarone toxicity
2. Amodiaquine hydrochloride administration
3. Atabrine administration
*4. Chloroquine and hydroxychloroquine toxicity
5. Chlorpromazine administration
*6. Fabry disease (diffuse angiokeratosis)
7. Incontinentia pigmenti
8. Indomethacin administration
9. Meperidine hydrochloride
10. Quinacrine administration
11. Urethan administration
Kaplan LJ, Cappaert WE. Amiodarone keratopathy. Arch Ophthalmol 1982; 100:601.
Ferreira RC, et al. Corneal abnormalities associated with incontinentia pigmenti. Am J

Opthalmol 1997; 123:549-551.
CORNEAL DERMOIDS
These congenital corneal limbal lesions grow slowly. Tumors are yellowish,
elevated, and variable in size; they consist of fibrofatty tissue covered by epidermal rather
than by conjunctival epithelium and may contain ectodermal derivatives such as hair
follicles, sebaceous glands, and sweat glands. Trauma, irritation, and puberty hasten their
growth.
1. Bloch-Sulzberger syndrome (incontinentia pigmenti)

2. Cri-du-chat syndrome (cat-cry syndrome)
3. Duane retraction syndrome
4. Multiple dermoids of the cornea associated with miliary aneurysms of the retina
5. Neurocutaneous syndrome (ectomesodermal dysgenesis)
6. Nevus sebaceous of Jadassohn (linear nevus sebaceous of Jadassohn)
*7. Oculoauriculovertebral dysplasia (Goldenhar syndrome)
9. Ring dermoid syndrome-autosomal dominant
10. Sporadic
11. Thalidomide teratogenicities
Benjamin SN, Allen HF. Classification for limbal dermoid choristomas and brachial arch
anomalies. Arch Ophthalmol 1972; 87:305-314.
Brodsky MC, et al. Oculocerebral dysgenesis in the linear nevus sebaceous syndrome.
Ophthalmology 1997; 104: 497-503.
Extracted Table Corneal Dermoids
CORNEAL PROBLEMS ASSOCIATED WITH KERATOTIC SKIN LESIONS

1. Ectodermal dysplasia (anhidrotic)
*2. Ichthyosis
3. Keratosis follicularis
4. Keratosis follicularis spinulosa decalvans
5. Keratosis plantaris and palmaris
6. Pityriasis rubra pilaris
CORNEAL PROBLEMS ASSOCIATED WITH LID EXCRESCENCES

1. Keratosis folliculosis
2. Lipid proteinosis
*3. Molluscum contagiosum
4. Verruca vulgaris
CORNEAL DISEASE ASSOCIATED WITH LENTICULAR PROBLEMS

1. Aberfeld syndrome (ocular and facial abnormalities syndrome) -cataracts, microcornea
2. Acrodermatitis chronica atrophicans-keratomalacia, corneal opacification, cataracts
3. Addison syndrome (idiopathic hypoparathyroidism) -keratoconjunctivitis, corneal
ulcers, keratitic moniliasis, cataracts
4. Amiodarone-corneal deposits, anterior subcapsular cataracts
5. Amyloidosis-amyloid deposits of cornea, corneal dystrophy, pseudopodia lentis
6. Anderson-Warburg syndrome (oligophrenia-microphthalmos syndrome)-corneal
opacification and lenticular destruction with a mass visible behind the lens
7. Andosky syndrome (atopic cataract syndrome)-atopic keratoconjunctivitis,
keratoconus, uveitis, subcapsular cataract
8. Aniridia-microcornea and subluxated lenses
9. Anterior chamber cleavage syndrome (Reese-Ellsworth syndrome) -corneal opacities,
anterior pole cataract
10. Anterior segment ischemia syndrome-corneal edema, corneal ulceration, cataract
11. Apert syndrome (absent digits cranial defects syndrome) -exposure keratitis,
cataracts, ectopia lentis
12. Arteriovenous fistula (arteriovenous aneurysm) -bullous keratopathy, cataract
13. Aspergillosis-corneal ulcer, keratitis, cataract
*14. Atopic disease (atopic eczema, Besnier prurigo)-keratoconjunctivitis, keratoconus,
cataract
15. Autosomal dominant-cataracts and microcornea
16. Avitaminosis C (scurvy)-keratitis, corneal ulcer, cataract
17. Chickenpox (varicella)-corneal ulcer, corneal opacity, keratitis, cataract
18. Chlorpromazine-corneal and lens opacities
19. Cholera-keratomalacia, cataract
20. Chromosome partial deletion (short-arm) syndrome-cataracts, corneal opacities
21. Cockayne syndrome (Mickey Mouse syndrome)-cataracts, band keratopathy, corneal
dystrophy
22. Congenital spherocytic anemia (congenital hemolytic jaundice) -congenital cataract,
ring-shaped pigmentary corneal deposits
23. Crouzon syndrome (Parrot-head syndrome) -exposure keratitis, cataract, corneal
dystrophy
24. Cryptophthalmia syndrome (cryptophthalmos-syndactyly syndrome) -cornea
differentiated from sclera, lens absence to hypoplasia, dislocation, and calcification
25. Cytomegalic inclusion disease (cytomegalovirus)-cataract, corneal opacities
26. Darier-White syndrome (keratosis follicularis)-keratosis, corneal subepithelial
infiltrations, corneal ulceration, cataract
27. Dermatitis herpetiformis (Duhring - Broca disease) -corneal vascularization, cataract
28. Diphtheria-keratitis, corneal ulcer, cataract
29. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)-microcornea,
keratoconus, lens subluxation
30. Electrical injury-corneal perforation, necrosis of cornea, anterior or posterior
subcapsular cataracts
31. Exfoliation syndrome (capsular exfoliation syndrome)-cataract, dislocated lens,
corneal dystrophy, lens capsule exfoliation
32. Folling syndrome (phenylketonuria)-corneal opacities, cataracts
*33. Fuchs syndrome (I) (heterochromic cyclitis syndrome)-secondary cataract,
edematous corneal epithelium
34. Goldscheider syndrome (epidermolysis bullosa)-bullous keratitis, corneal
subepithelial blisters to corneal perforation, cataract
35. Gorlin-Goltz syndrome (multiple basal cell nevi syndrome)-cataract, corneal leukoma
36. Grönblad-Strandberg syndrome (elastorrhexis)-keratoconus, cataract, subluxation of
lens
37. Hallermann-Streiff syndrome (oculomandibulofacial dyscephaly)-cataracts,
microcornea
38. Hanhart syndrome (recessive keratosis palmoplantaris)-dendritic corneal lesions,
keratitis, corneal haze, corneal neovascularization, cataract
39. Heerfordt syndrome (uveoparotid fever)-band keratopathy, keratoconjunctivitis,
cataract
40. Hereditary ectodermal dysplasia syndrome (Siemens syndrome)-keratosis, corneal
erosions, corneal dystrophy, cataract, lens luxation
*41. Herpes simplex-keratitis, corneal ulcer, cataract
*42. Herpes zoster-keratitis, corneal ulcer, cataract
43. Histiocytosis X (Hand-Schüller-Christian syndrome)-pannus, bullous keratopathy,
corneal ulcer, cataract
44. Hodgkin disease-keratitis, cataract
45. Homocystinuria syndrome-dislocated lens, cataract, keratitis
46. Hutchinson-Gilford syndrome (progeria)-cataract, microcornea
47. Hydatid cyst (echinococcosis)-keratitis, corneal abscess, cataract
48. Hypervitaminosis D-band keratopathy, cataract
49. Hypoparathyroidism-keratitis, cataract
50. Hypophosphatasia (phosphoethanolaminuria)-cataract, corneal subepithelial
calcifications
51. Influenza-keratitis, cataract
52. Jadassohn-type anetodermal-keratoconus, cataract
53. Jadassohn-Lewandowsky syndrome (pachyonychia congenita)-corneal dyskeratosis,
cataract
*54. Juvenile rheumatoid arthritis (Still disease)-band keratopathy, cataract
55. Kussmaul disease (periarteritis nodosa)-corneal ulcer, cataract
56. Kyrle disease (hyperkeratosis penetrans)-subcapsular cataracts, subepithelial corneal
opacities
57. Leri syndrome (carpal tunnel syndrome)-corneal clouding, cataract
58. Listerellosis (listeriosis)-keratitis, corneal abscess and ulcer, cataract
59. Little syndrome (nail-patella syndrome)-microcornea, keratoconus, cataract
60. Lowe syndrome (oculocerebrorenal syndrome) -cloudy cornea, cataracts,
megalocornea, corneal dystrophy
61. Miliaria-keratitis, cataract
62. Marchesani syndrome (brachymorphia with spherophakia) -lentic ulnar myopia,
ectopia lentis, megalocornea, corneal opacity
63. Marfan syndrome (arachnodactyly-dystrophia-mesodermalis congenita)-lens
dislocation, cataract, megalocornea, lenticular myopia
64. Matsoukas syndrome (oculocerebroarticuloskeletal syndromes)-cataract, corneal
sclerosis
65. Meckel syndrome (dysencephalia splanchnocystica syndrome) -sclerocornea,
microcornea, cataract
66. Morbilli (rubeola, measles)-corneal ulcer, cataract
67. Mucolipidosis IV (ML IV)-corneal clouding, cataract
68. Nematode ophthalmia syndrome (toxocariasis)-cataract, larvae present in the cornea
69. Neurodermatitis (lichen simplex chronicus)-keratoconjunctivitis, atopic cataracts,
keratoconus
70. Ocular toxoplasmosis (toxoplasmosis)-keratitis, cataract
71. Oculodental syndrome (Peter syndrome)-macrocornea, opacities of the corneal
margin, ectopic lentis, corneoscleral staphyloma
72. O'Donnell-Pappas syndrome-presenile cataract, peripheral corneal pannus
73. Paget syndrome (osteitis deformans) -corneal ring opacities, cataract
74. Passow syndrome (status dysraphicus syndrome) -neuroparalytic keratitis, zonular
cataract
75. Pemphigus foliaceus (Cazenave disease)-pannus, corneal infiltration, cataract
76. Pigmentary ocular dispersion syndrome (pigmentary glaucoma)-Krukenberg spindle,
equatorial pigmentation of lens capsule
77. Pseudohypoparathyroidism (Seabright-Bantan syndrome) -punctate cataracts, keratitis
78. Radiation-corneal ulcer, punctate keratitis, cataracts, exfoliation of lens capsule
79. Refsum syndrome (phytanic acid oxidase deficiency) -corneal opacities, cataracts
80. Relapsing polychondritis-corneal ulcer, cataracts, keratoconjunctivitis sicca
81. Retinal disinsertion syndrome-lens subluxation, keratoconus
82. Retinopathy of prematurity-cataracts, corneal opacification
83. Rieger syndrome (dysgenesis mesodermalis corneae et irides)-microcornea, corneal
opacities in Descemet membrane, dislocated lens
84. Romberg syndrome (facial hemiatrophy)-neuroparalytic keratitis, cataracts
85. Rubella syndrome (German measles)-corneal haziness, cataracts, microcornea
86. Sabin-Feldman syndrome-posterior lenticonus, microcornea
87. Sanfilippo-Good syndrome (MPS III)-deposits in cornea and lens
88. Schafer syndrome (keratosis palmoplantaris syndrome)-lesions in the lower portion of
the cornea, cataract
89. Schaumann syndrome (sarcoidosis syndrome)-keratitis sicca, band-shaped keratitis,
complicated cataract
90. Scheie syndrome (MPS IS)-diffuse haze to marked corneal clouding, cataracts
91. Stannus cerebellar syndrome-corneal vascularization, corneal opacities, cataracts
92. Stevens-Johnson syndrome (erythema multiforme exudativum)-keratitis, corneal
ulcers, cataracts, pannus
93. Stickler syndrome (hereditary progressive arthroophthalmopathy)-keratopathy,
cataracts
94. Thioridazine-corneal and lens opacities
95. Toxic lens syndrome-pigment precipitation on the surface of an intraocular lens,
chronic uveitis
96. Trisomy syndrome-corneal and lens opacities
97. Turner syndrome (gonadal dysgenesis )-corneal nebulae, cataracts
98. Ultraviolet radiation-band keratopathy, keratitis, discoloring of lens
99. van Bogaert-Scherer-Epstein syndrome (familial hypercholesterolemia syndrome)-
lipid keratopathy, cataract, juvenile corneal arcus
100. von Recklinghausen syndrome (neurofibromatosis)-nodular swelling of corneal
nerves, cataracts
101. Waardenburg syndrome (interoculoiridodermatoauditive dysplasia)-microcornea,
cornea plana, lenticonus
102. Wagner syndrome (hereditary hyaloideoretinal degeneration and palatoschisis)-
corneal degeneration, including band-shaped keratopathy, cataracts
103. Ward syndrome (nevus jaw cyst syndrome)-congenital cataracts, congenital corneal
opacities
104. Wegener syndrome (Wegener granulomatosis)-corneal ulcer, corneal abscess,
cataract
105. Weil disease (leptospirosis)-keratitis, cataract
106. Werner syndrome (progeria of adults)-juvenile cataracts, bullous keratitis, trophic
corneal defects
107. Yersiniosis-corneal ulcer, cataract
108. Zellweger syndrome (cerebrohepatorenal syndrome of Zellweger)-corneal opacities,
cataract
Bilgami NL, et al. Marfan syndrome with microcornea, aphakia, and ventricular systolic
defect. Indian Heart J 1981; 33:78-80.
Gualtieri CT, et al. Corneal and lenticular opacities in mentally retarded young adults
treated with thioridazine and chlorpromazine. Am J Psychiatry 1982; 139:1178-1180.
Polomeno RC, Cummings C. Autosomal dominant cataracts and microcornea. Can J

Ophthalmol 1979; 14:227-229.
CORNEAL DISEASE ASSOCIATED WITH RETINAL PROBLEMS

1. Abdominal typhus (enteric fever)-corneal ulcer, retinal detachment, central retinal
artery emboli
2. Acanthamoeba-keratitis, pannus, corneal ring abscess, retinal perivasculitis
3. African eyeworm disease-keratitis, central retinal artery occlusion, macular
hemorrhages
4. Amyloidosis-amyloid corneal deposits, corneal dystrophy, retinal hemorrhages
5. Anderson- Warburg syndrome (oligophrenia-microphthalmos syndrome) -corneal
opacification, malformed retina with retina pseudotumors
6. Angioedema (hives)-central serous retinopathy, corneal edema
7. Anterior segment ischemia syndrome-corneal edema midperiphery retinal hemorrhages
8. Apert syndrome (acrodysplasia)-exposure keratitis, retinal detachment
9. Arteriovenous fistula-bullous keratopathy, retinal hemorrhages
10. Aspergillosis-corneal ulcer, keratitis, retinal hemorrhages, retinal detachment
11. Atopic dermatitis-keratoconus and retinal detachment
12. Avitaminosis C-retinal hemorrhages, keratitis, corneal ulcer
13. Bacillus cereus-ring abscess of cornea, necrosis of retina
14. Bang disease (brucellosis)-keratitis, chorioretinitis, macular edema
15. Behçet syndrome (dermatostomata-ophthalmic syndrome)-keratitis, posterior corneal
abscess, retinal vascular changes
16. Bietti disease (Bietti marginal crystalline dystrophy)-marginal corneal dystrophy,
retinitis punctate albescens
17. Candidiasis-keratitis, corneal ulcer, retinal atrophy, retinal detachment
18. Carotid artery syndrome-corneal ulcer, loss of corneal sensation, retinal edema,
engorgement of retinal veins
19. Chickenpox (varicella)-corneal ulcer, corneal opacity, retinitis, hemorrhagic
retinopathy
*20. Chloroquine-corneal epithelial pigmentation, macular lesions
21. Chronic granulomatous disease of childhood-keratitis, destructive chorioretinal
lesions
22. Cockayne syndrome (dwarfism with retinal atrophy and deafness)-pigmentary
degeneration, band keratopathy, corneal dystrophy
23. Crohn disease (granulomatous ileocolitis)-marginal corneal ulcers, keratitis, macular
edema, macular hemorrhages
24. Cryoglobulinemia-deep corneal opacities, venous stasis
25. Cystinosis (aminoaciduria)-crystals in cornea and pigment in retina
26. Dengue fever-keratitis, corneal ulcer, retinal hemorrhages
27. Diffuse keratoses syndrome-corneal nodular thickening in the stroma worse in fall,
retinal phlebitis
28. Diphtheria-keratitis, corneal ulcer, central artery occlusion
29. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)-keratitis,
keratoconjunctivitis sicca, corneal ulcer, central retinal vein occlusion, retinal
detachment
30. Ehlers-Danlos syndrome (cutis hyperelastica)-keratoconus and retinitis pigmentosa
31. Electrical injury-corneal perforation, retinal edema, retinal hemorrhages, pigmentary
degeneration, retinal holes, dilatation of retinal veins
32. Fabry disease (diffuse angiokeratosis)-whorl-like changes in cornea, central retinal
artery occlusion, tortuosity of retinal vessels
33. Goldscheider syndrome (epidermolysis bullosa)-bullous keratitis with opacities,
retinal detachment
34. Gronblad-Strandberg syndrome (systemic elastodystrophy)-angioid streaks of the
retina, macular hemorrhages, retinal detachment, keratoconus
35. Hamman-Rich syndrome (alveolar capillary block syndrome)-keratomalacia ischemic
retinopathy, cystic macular changes
36. Heerfordt syndrome (uveoparotid fever)-band keratopathy, retinal vasculitis
37. Hennebert syndrome (luetic otitic nystagmus syndrome)-interstitial keratitis,
disseminated syphilitic chorioretinitis
38. Histiocytosis X (Hand-Schüller-Christian syndrome)-retinal hemorrhage, retinal
detachment, bullous keratopathy, corneal ulcer, pannus
39. Hodgkin disease-keratitis, retinal hemorrhages
40. Hollenhorst syndrome (chorioretinal infarction syndrome)-hazy cornea, serous retinal
detachment, pigmentary retinopathy
41. Hunter syndrome (MPS II)-splitting or absence of peripheral Bowman membrane,
stromal haze, pigmentary retinal degeneration, narrowed retinal vessels
42. Hurler-Scheie syndrome (MPS IH-S)-corneal clouding, pigmentary retinopathy
43. Hurler syndrome (gargoylism)-diffuse corneal haziness, retinal pigmentary changes,
megalocornea, retinal detachment
44. Hydatid cyst (echinococcosis)-keratitis, abscess of cornea, retinal detachment, retinal
hemorrhages
45. Hyperlipoproteinemia-arcus juvenilis, lipemia retinalis, xanthomata of retina
46. Hyperparathyroidism-band keratopathy, vascular engorgement of retina
47. Hypovitaminosis A-keratomalacia with perforation, corneal opacity, retinal
degeneration
48. Idiopathic hypercalcemia (blue-diaper syndrome)-band keratopathy, optic atrophy,
papilledema
49. Indomethacin-corneal deposits, reduced retinal sensitivity
50. Influenza-keratitis, retinal hemorrhage
51. Japanese River fever (typhus)-keratitis, retinal hemorrhages
52. Juvenile rheumatoid arthritis (Still disease)-band keratopathy, macular edema
53. Kahler disease (multiple myeloma)-crystalline deposits of cornea, central retinal
artery occlusion, retinal microaneurysms
54. Kussmaul disease (periarteritis nodosa)-retinal detachment, pseudoretinitis
pigmentosa, corneal ulcer
55. Leber tapetoretinal dystrophy syndrome (retinal aplasia)-keratoconus, salt-and-pepper
or "bone corpuscle" pigmentation, yellowish-brown or gray macular lesions
56. Lubarsch-Pick syndrome (primary amyloidosis)-amyloid corneal deposits, retinal
hemorrhages
57. Lymphogranuloma venereum disease (Nicolas-Favre disease)-keratitis, pannus,
corneal ulcer, keratoconus, tortuosity of retinal vessels, retinal hemorrhages
58. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)-keratoconus,
retinitis pigmentosa
59. Meckel syndrome (dysencephalia splanchnocystica syndrome) -sclerocornea,
microcornea, retinal dysplasia
60. Meningococcemia-keratitis, retinal endophlebitis
61. Mikulicz-Radeski syndrome (dacryosialoadenopathy)-keratoconjunctivitis, retinal
candlewax spots
62. ML IV (mucolipidosis IV)-corneal clouding, corneal opacities, retinal atrophy
63. Morbilli (measles-rubeola)-keratitis, corneal ulcer, pigmentary retinopathy, central
retinal artery occlusion
64. Mucormycosis (phycomycosis)-corneal ulcer, striate keratopathy, retinitis, central
retinal artery thrombosis
65. Mycosis fungoides syndrome (malignant cutaneous reticulosis syndrome)-
keratoconjunctivitis, retinal edema, retinal hemorrhage
66. Myotonic dystrophy syndrome-corneal epithelial dystrophy, loss of corneal
sensitivity, tapetoretinal degeneration, macular red spot, macular degeneration,
chorioretinitis
67. Neurofibromatosis (von Recklinghausen syndrome)-nodular swelling nerves,
hamartoma of retina
68. Norrie disease (atrophia oculi congenita)-malformation of sensory cells of retina,
corneal nebulae
69. Oculodental syndrome (Peter syndrome)-corneoscleral staphyloma, megalocornea,
corneal marginal opacities, macular pigmentation
70. Onchocerciasis syndrome-punctate keratitis, sclerosing keratitis, chorioretinitis,
retinal degeneration
71. Paget syndrome (osteitis deformans)-corneal ring opacities, retinal hemorrhages,
pigmentary retinopathy, macular changes resembling Kuhnt-Junius degeneration
72. Phenothiazine-epithelial and endothelial pigment, retinal pigmentation
73. Pierre Robin syndrome (micrognathia-glossoptosis)-retinal disinsertion,
megalocornea
74. Plasma lecithin (cholesterol acyltransferase deficiency)-corneal stromal opacities,
retinal hemorrhages
75. Porphyria cutanea tarda-keratitis, retinal hemorrhages, cotton-wool spots, macular
edema
76. Postvaccinial ocular syndrome-corneal vesicles, and marginal ulcers, chorioretinitis,
central serous retinopathy, central retinal vein thrombosis
77. Progressive systemic sclerosis-marginal corneal ulcers with cicatrization, cotton-wool
spots, retinal hemorrhages
78. Radiation-corneal ulcer, punctate keratitis, keratoconjuctivitis sicca, retinal
hemorrhage, macular degeneration, macular holes with vascularization
79. Refsum syndrome (phytanic acid oxidase deficiency)-band keratopathy, retinitis
pigmentosa
80. Relapsing fever-interstitial keratitis, retinal hemorrhage
81. Relapsing polychondritis-corneal ulcer, retinal detachment, retinal artery thrombosis,
keratoconjunctivitis sicca
82. Renal failure-cotton-wool spots, band keratopathy
83. Rendu-Osler syndrome (hereditary hemorrhagic telangiectasis)-intermittent
filamentary keratitis, small retinal angiomas, retinal hemorrhages
84. Retinal disinsertion syndrome-bilateral keratoconus, retinal detachment
85. Retinoblastoma-corneal tumor, retinal neovascularization
86. Rothmund syndrome (telangiectasia-pigmentation cataract syndrome)-corneal lesions,
retinal hyperpigmentation
87. Rubella syndrome (Gregg syndrome)-microcornea, pigmentary retinal changes
88. Sabin-Feldman syndrome-microcornea, chorioretinitis or atrophic degenerative
chorioretinal changes
89. Sanfilippo-Good syndrome (MPS III)-slight narrowing of retinal vessels, acid
mucopolysaccharide deposits in cornea
90. Schaumann syndrome (sarcoidosis syndrome)-mutton fat keratitic precipitates,
keratitis sicca, band-shaped keratitis, inflammatory retinal exudates
91. Scheie syndrome (MPS IS)-diffuse to marked corneal clouding, tapetoretinal
degeneration
92. Schwartz syndrome (glaucoma associated with retinal detachment)-retinal
detachment, microcornea
93. Shy-Gonatas syndrome (orthostatic hypotension syndrome)-keratopathy, corneal
ulcer, lattice-like white opacities in the area of Bowman membrane, retinal
pigmentary degeneration
94. Smallpox-keratitis, congenital corneal clouding, chorioretinitis
95. Stannus cerebellar syndrome (riboflavin deficiency)-corneal vascularization,
superficial diffuse keratitis, corneal opacities, brownish retinal patches
chorioretinal degeneration, total retinal detachment
97. Sturge-Weber syndrome (neurooculocutaneous angiomatosis) -retinal detachment,
increased corneal diameter with cloudiness
98. Syphilis (acquired lues)-keratitis, retinal hemorrhages, retinal proliferation
99. Temporal arteritis syndrome (Hutchinson-Horton-Magath-Brown syndrome)-retinal
detachments, narrowing of retinal vessels, central retinal artery occlusion, corneal
hypesthesia
100. Trisomy 13 (Patau syndrome)-malformed cornea, retinal dysplasia
101. Tuberculosis-keratitis, pannus, corneal ulcer, retinitis
102. Ullrich syndrome (dyscraniopygophalangy)-cloudy cornea, corneal ulcers,
chorioretinal coloboma
103. Ultraviolet radiation-photokeratitis, band keratopathy, herpes simplex keratitis,
recurrent corneal erosions, retinal degeneration
104. Vaccinia-keratitis, pannus, corneal perforation, central serous retinopathy,
pseudoretinitis pigmentosa
105. van Bogaert -Scherer-Epstein (primary hyperlipidemia) -arcus juvenilis of the
cornea, lipid keratopathy, retinopathy with yellowish deposits
106. Vitreous tug syndrome-vitreous strands attached to corneal wound or scar,
circumscribed retinal edema, posterior retinal detachment
107. von Gierke disease (glycogen storage disease type I)-corneal clouding, discrete non-
elevated, yellow flecks in macula
108. Waardenburg syndrome (embryonic fixation syndrome)-microcornea, cornea plana,
hypopigmentation and hypoplasia of retina
109. Wagner syndrome (hyaloideoretinal degeneration)-corneal degeneration, band-
shaped keratopathy, hyaloideoretinal degeneration, narrowing of retinal vessels,
retinal detachment, avascular preretinal membranes
110. Waldenström syndrome (macroglobulinemia syndrome)-crystalline corneal deposits,
keratoconjunctivitis sicca, retinal venous thrombosis, retinal microaneurysms,
cotton-wool spots
111. Weil disease (leptospirosis)-keratitis, retinitis
112. Werner syndrome (progeria of adults) -bullous keratitis, paramacular retinal
degeneration
113. Wiskott-Aldrich syndrome (sex-linked draining ears, eczematoid, dermatitis, bloody
diarrhea)-corneal ulcers, retinal hemorrhages
114. Yersiniosis-corneal ulcer, retinal hemorrhages
115. Zellweger syndrome (cerebrohepatorenal syndrome)-corneal opacities, narrowing of
retinal vessels, retinal holes without detachment, tapetoretinal degeneration
116. Zieve syndrome (hyperlipemia hemolytic anemia-icterus syndrome)-cloudy cornea,
corneal ulcers, retinal lipemia
Arffa RC. Grayson's disease of the cornea, 3rd ed. St. Louis: Mosby- Year Book, 1991.
CORNEAL DISEASES ASSOCIATED WITH DEAFNESS

1. Atopic dermatitis-limbal keratitis, conjunctivitis
*2. Cogan syndrome (nonsyphilitic interstitial keratitis)-interstitial keratitis
3. Meniere disease-iritis, glaucoma
*4. Polyarteritis nodosa (Kussmaul disease)-paralimbal keratitis, corneoscleral ulceration
5. Sarcoidosis syndrome (Schaumann syndrome)-primary stromal keratitis,
*6. Syphilis (lues)-interstitial keratitis
7. 3-methyl-pentyn-3-yl acid phthalate (Whipcide, trichuricidal agent)-keratitis, uveitis,
stromal keratitis
8. Tuberculosis-interstitial keratitis
9. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia poliosis syndrome) -uveitis
10. Wegener granulomatosis-necrotizing sclerokeratitis
Heinemann MH, et al. Cogan's syndrome. Ann Ophthalmol 1980; 12:667.
TRIGGER MECHANISMS FOR RECURRENT HERPES SIMPLEX KERATITIS

*1. Corticosteroids (topical)
*2. Emotional disturbances
*3. Exposure to sunlight (ultraviolet)
*4. Fever (most common)
5. Gastrointestinal upsets
6. Ingestion of food to which patient is allergic
7. Mechanical trauma
8. Menses
Kimura SJ. Infectious diseases of the conjunctiva and cornea. Symposium of the New
Orleans Academy of Ophthalmology. St. Louis: CV Mosby, 1963.
PREDISPOSING FACTORS IN KERATOMYCOSIS

1. Antibiotics
2. Steroids
3. Trauma
Francois J, Ryssdlaere M. Oculomycoses. Springfield, IL: Charles C Thomas, 1972.
Gingrich WD. Infectious diseases of the conjunctiva and cornea. Symposium of the New
Orleans Academy of Ophthalmology. St. Louis: CV Mosby, 1963:154.
SCLEROCORNEA
This type of developmental corneal abnormality has ill-defined limbus due to
extension of opaque scleral tissue into the cornea. Vision varies with involvement.
Somatic abnormalities include craniofacial, digital, skin, and testis abnormalities;
deafness and mental retardation are seen.
1. Associated ocular abnormalities including:

A. Abnormalities of Descemet membrane, endothelium, and corneal stroma
B. Aniridia
C. Cataract
D. Coloboma
E. Cornea plana-occurrence
F. Dysgenesis of angle and iris
G. Esotropia
H. Glaucoma
I. Iridocorneal synechiae
J. Microphthalmia
K. Nystagmus
L. Persistent pupillary membrane
M. Posterior embryotoxon
2. Associated syndromes, including the following:
A. Axenfeld syndrome
B. Cross syndrome
C. Dandy-Walker syndrome
D. Hallermann-Streiff syndrome
E. Hurler syndrome
F. Hypomelanosis of Ito
G. Lobstein syndrome
H. Melnick-Needles syndrome
I. Mieten syndrome
J. Nail-patella syndrome
K. Rieger syndrome
L. Robert syndrome
M. Smith-Lemli-Opitz syndrome
N. Unbalanced 17p-10q translocation
O. Wolf syndrome (4p-syndrome)

1990.
POSTOPERATIVE CORNEAL MELT

This condition is characterized by central or peripheral thinning of corneal stroma
after pterygium, refractive surgery, keratoplasty, glaucoma surgery, strabismus surgery,
cataract surgery, or retina surgery.
1. AIDS
2. Collagen disorders
a. Polyarteritis nodosa
b. Rheumatoid arthritis
c. Scleroderma
d. Systemic lupus erythematosus
3. Delayed corneal wound healing due to drugs, including adenine arabinoside, adrenal
cortex injections, aldosterones, alpha-chymotrypsin, azathioprine, betarnethasone,
butacaine, cocaine, cortisone, dexamethasone, fluorometholone, fluprednisolone,
hydrocortisone, prednisolone, sulfacetamide, sulfamethizole, tetracaine, triamcinolone
4. Drug use
a. Steroid therapy
b. Stevens-Johnson syndrome
c. Topical anesthesia abuse
d. Topical nonsteroidal antiinflammatory agents
5. Marginal ring ulcer
a. Acute leukemia
b. Bacillary dysentery
c. Brucellosis
d. Dengue fever
e. Gonococcal arthritis
f. Hookworm infestation
g. Influenza
h. Possibly porphyria trigger mechanisms
6. Ocular predisposing factors
a. Phlyctenular keratoconjunctivitis
b. Superior limbic keratoconjunctivitis
c. Trachoma
d. Terrien disease
e. Vernal conjunctivitis
7. Preexisting corneal dellen
8. Skin conditions
a. Acne rosacea
b. Benign mucosa; pemphigoid
c. Dermatitis herpetiformis
d. Ectodermal dysplasia
e. Herpes zoster
f. Ichthyosis
g. Incontinentia pigment
h. Leprosy
i. Lichen planus
j. Lyell disease
k. Lymphopathia venereum
l. Molluscum contagiosum
m. Palmoplantar keratosis
n. Pemphigus foliaceus
o. Pityriasis rubra pilaris
p. Psoriasis
9. Systemic diseases
a. Brucellosis
b. Deerfly fever
c. Filariasis
d. Gout
e. H. influenzae
f. Hodgkin disease
g. Hypoparathyroidism
h. Leprosy
i. Lymphopathia venereum
j. Mumps
k. Sarcoidosis
l. Trypanosomiasis
m. Tuberculosis
n. Upper respiratory infection
10. Vitamin deficiency
a. Ariboflavinosis keratopathy
b. Pellagra
c. Vitamin A deficiency
d. Vitamin B2 deficiency
Castillo A, et al. Peripheral melt of flap after laser in situ keratomileusis. J Refract Surg
1998; 14:61-63.
Liu SM, et al. Corneal melting after avulsion of a Molteno shunt. J Glaucoma 1997;
6:357-358.
Wilhelmus KR. Corneal edema following argon laser iridotomy. Ophthalmic Surg Lasers
1992; 23:533-537.
CORNEAL MUCOUS PLAQUES

These plaques are abnormal collections of a mixture of mucous, epithelial cells,
and proteinaceous and lipoidal material that adhere firmly to the corneal surface.
1. Local radiation exposure

2. Herpes zoster
*3. Keratoconjunctivitis sicca
4. Other forms of keratitis
5. Vernal keratoconjuctivitis
Fraunfelder Fr, et al. Corneal mucous plaques. Am J Ophthalmol 1977; 83:31-35.
Tripathi RC, et al. Contact lens deposits and spoilage: identification and management. Int
Ophthalmol Clin 1991; 31:91-120.
9
Intraocular Pressure
CONTENTS
Glaucoma suspect, infant 305
Conditions simulating congenital glaucoma 305
Syndromes and diseases associated with glaucoma 307
Glaucoma suspect, adult 312
Elevated intraocular pressure measurement with normal-appearing optic disc 313
Secondary open-angle glaucoma 314
Unilateral glaucoma 315
Glaucoma associated with displaced lens 317
Glaucoma and elevated episcleral venous pressure 318
Glaucoma associated with shallow anterior chamber 318
Glaucoma in aphakia or pseudophakia 319
Medications and chemicals that may cause elevated intraocular pressure 320
Primary low-tension glaucoma 323
Neovascular glaucoma 324
Hypotony 325
Glaucoma associated with uveitis 326
Glaucoma associated with intraocular tumors 327
GLAUCOMA SUSPECT, INFANT
1. Amblyopia ex anopsia
*2. Corneal edema (see p. 250)
3. Corneal enlargement
4. Cupping and atrophy of optic disc
5. Deep anterior chamber
*6. Epiphora, photophobia, and blepharospasm (see p. 65-66)
7. Iridodonesis and subluxation of lens (see p. 374)
8. Iris processes
*9. Tears in Descemet membrane (see p. 287)
Duane TD, Jaeger EA. Clinical ophthalmology. New York: Harper & Row, 1994.
Hoskins HD, Kass MA. Becker-Shaffer's diagnosis and therapy of glaucoma, 6th ed. St.
Louis: CV Mosby, 1989.
Shields MB. Textbook of glaucoma, 3rd ed. Baltimore: Williams & Wilkins, 1992.
CONDITIONS SIMULATING CONGENITAL GLAUCOMA
1. Blue sclera
A. Albright hereditary osteodystrophy (pseudohypoparathyroidism)
B. Craniofacial dysostosis (Crouzon disease)
C. de Lange syndrome
D. Ehlers-Danlos syndrome
E. Hilling syndrome
F. Hallermann-Streiff syndrome (oculomandibulodyscephaly)
G. Incontinentia pigmenti (Bloch-Sulzberger syndrome)
H. Juvenile Paget disease (hyperphosphatasia, hereditary)
I. Lowe (oculocerebrorenal) syndrome
J. Marfan syndrome
K. Turner (XO, gonadal dysgenesis) syndrome
L. van der Hoeve syndrome (osteogenesis imperfecta)
M. Werner syndrome
2. Corneal opacity
A. Congenital malformations
(1) Anterior corneal staphyloma
(2) Cornea plana
(4) Norrie disease
*(5) Peters anomaly
(6) Riley-Day syndrome (congenital familial dysautonomia)
*(7) Sclerocornea
(8) Trisomy 13-15 syndrome (Patau syndrome)
B. Edema
*(1) Birth injury, such as breaks in Descemet membrane
(2) Congenital hereditary corneal edema
(3) Fetal uveitis
*(4) Infectious keratitis (congenital syphilis, interstitial keratitis, rubella,
variola, varicella, gonorrhea, mumps, and others)
(5) Keratitis (chemical)
*(6) Keratoconus
C. Metabolic disorders/dystrophies
(1) Congenital hereditary stromal dystrophy
(2) Corneal amyloidosis (Lubarsch-Pick syndrome)
(3) Corneal lipidosis
(4) Cystinosis (Lignac-Fanconi syndrome)
(5) Fabry disease
(6) Hyperlipidemia
(7) Mucopolysaccharidoses (MPS)
a. Hunter syndrome (MPS IIB)
b. Hurler syndrome (MPS IH)
c. Maroteaux-Lamy syndrome (MPS VI)
d. Morquio syndrome (MPS IV)
e. Sanfilippo syndrome (MPS IIIC)
f. Scheie syndrome (MPS IS)
(8) Porphyria (congenital)
(9) von Gierke glycogen storage disease
3. Epiphora (excessive tearing)
*A. Lacrimal duct obstruction
*B. Viral, chemical or allergic conjunctivitis
4. Large corneas
A. Apert syndrome
B. High myopia
C. Keratoglobus
D. Marfan syndrome
E. Megalocomea
F. van der Hoeve syndrome (osteogenesis imperfecta)
*5. Photophobia-anterior uveitis (many causes)
Teekhasaenee C, Ritch R. Iridocorneal endothelial syndrome in Thai patients. Arch

Ophthalmol 2000; 118: 187 -192.
SYNDROMES AND DISEASES ASSOCIATED WITH GLAUCOMA
1. Ocular disease
A. Corneal endothelial disorders
(1) Fuchs endothelial dystrophy
(2) Iridocorneal endothelial (ICE) syndrome
a. Chandler syndrome
b. Cogan-Reese (iris-nevus) syndrome
c. Progressive iris atrophy
(3) Posterior polymorphous dystrophy
B. Developmental glaucoma with associated ocular anomalies
(1) Aniridia
(2) Axenfeld-Rieger syndrome
(3) Congenital ectropion uveae
(4) Congenital iris hypoplasia
(5) Megalocornea
(6) Microcornea
(7) Peters anomaly
(8) Sclerocornea
C. Elevated episcleral venous pressure (see p. 235)
D. Iris disorders
(1) Iridoschisis
*(2) Pigmentary glaucoma
E. Lens disorders
(1) Cataract
a. Lens-particle glaucoma
b. Phacoanaphylaxis
c. Phacolytic (lens protein) glaucoma
d. Phacomorphic (intumescent lens) glaucoma
(2) Dislocation of the lens
*(3) Exfoliation syndrome
F. Medications or chemicals
(1) Corticosteroids
(2) Others
G. Myopia
H. Ocular hemorrhage
(1) Degenerated ocular blood
a. Ghost cell glaucoma
b. Hemolytic glaucoma
c. Hemosideric glaucoma
*(2) Hyphema
a. Blunt trauma
b. Intraocular surgery
1. Intraoperative
2. Postoperative
c. Penetrating trauma
d. Spontaneous
1. Anterior segment neovascularization
2. Intraocular tumor
3. Pupillary vascular tufts
(3) Orbital hemorrhage (massive)
(4) Vitreous hemorrhage (massive)
I. Ocular inflammation
(1) Choroiditis and retinitis
a. Cytomegalic inclusion retinitis
b. Sympathetic ophthalmia
c. Toxocariasis
d. Vogt-Koyanagi-Harada syndrome
(2) Episcleritis
*(3) Iridocyclitis
a. Acute anterior iridocyclitis
b. Ankylosing spondylitis
c. Behçet disease
d. Fuchs heterochromic cyclitis
e. Glaucomatocyclitic crisis (Posner-Schlossman syndrome)
f. Infectious diseases
1. Acquired immunodeficiency syndrome (AIDS)
2. Congenital rubella
3. Disseminated meningococcemia
4. Hansen disease (leprosy)
5. Hemorrhagic fever with renal syndrome
6. Onchocerciasis (also keratitis)
7. Syphilis
g. Juvenile rheumatoid arthritis (JRA)
h. Pars planitis
i. Precipitates on the trabecular meshwork (Grant syndrome)
j. Reiter syndrome
k. Sarcoid
l. Trauma
(4) Keratitis
a. Adenovirus type 10
b. Herpes simplex
c. Herpes zoster
d. Interstitial keratitis
(5) Scleritis
*J. Ocular surgery
(1) Aphakia or pseudophakia (see p. 405)
(2) Corticosteroid induced
(3) Cyclodialysis cleft (sudden closure)
(4) Epithelial downgrowth
(5) Malignant (ciliary block) glaucoma
(6) Penetrating keratoplasty
a. Distortion of angle structures
b. Graft rejection
(7) Vitreoretinal procedures
a. Intravitreal gas
b. Pars plana vitrectomy
c. Retinal photocoagulation
d. Scleral buckling surgery
e. Silicone oil
K. Ocular trauma
(1) Chemical bums (acid, alkali, and others)
(2) Contusion injuries
a. Angle recession
b. Hyphema
c. Iritis
d. Lens damage or dislocation
e. Trabecular damage
(3) Penetrating injuries
a. Epithelial downgrowth
b. Hyphema
c. Lens damage or dislocation
d. Peripheral anterior synechiae
(4) Radiation damage
(5) Retained intraocular foreign body (iron, copper)
(6) Retrobulbar hemorrhage (massive)
L. Ocular tumors
(1) Benign tumors of the anterior uvea
a. Adenomas
b. Cysts (primary versus secondary)
c. Iris nevi
d. Leiomyomas
e. Melanocytomas
f. Melanoses
(2) Histiocytosis X
(3) Leukemias
(4) Lymphomas
(5) Metastatic tumors
a. Carcinomas (most commonly, breast carcinoma in females and
lung carcinoma in males)
b. Melanomas
(6) Multiple myeloma
(7) Ocular tumors of childhood
a. Juvenile xanthogranuloma
b. Medulloepithelioma (diktyoma)
c. Retinoblastoma
(8) Orbital tumors
(9) Primary uveal melanomas
(10) Retrobulbar tumors
M. Retinal, vitreous, and choroidal disorders
(1) Angle closure
a. Acute choroidal hemorrhage
b. Central retinal vein occlusion (CRVO)
c. Ciliochoroidal effusion
1. AIDS
2. Arteriovenous malformations
3. Inflammatory conditions
4. Nanophthalmos
5. Surgery
6. Trauma
7. Tumors
8. Uveal effusion syndrome
d. Hemorrhagic retinal and choroidal detachment
e. Iris retraction syndrome with retinal detachment (Campbell)
f. Persistent hyperplastic primary vitreous (PHPV)
g. Postoperative panretinal photocoagulation
h. Postoperative scleral buckle
i. Retinal dysplasia
j. Retinopathy of prematurity (retrolental fibroplasia)
*(2) Neovascular glaucoma (see p. 324)
(3) Retinitis pigmentosa
(4) Rhegmatogenous retinal detachment (Schwartz syndrome)
2. Systemic disorders
A. AIDS
B. Angioneurotic edema (giant urticaria)
C. Ankylosing spondylitis (Marie-Strümpell disease)
D. Aortic arch syndrome
E. Behçet disease
F. Carotid artery occlusive disease
G. Carotid-cavernous fistula
H. Cavernous sinus thrombosis
I. Crouzon disease (craniofacial dysostosis)
J. Cushing disease
K. Developmental glaucoma as part of a syndrome
(1) Bing-Neel [macroglobulinemia and central nervous system (CNS)]
syndrome
(2) Chondrodystrophy, joint dislocation, glaucoma, and mental retardation
(3) Chromosomal abnormalities
a. Chromosome partial deletion (long-arm) syndrome
b. Pericentric inversion of chromosome II
c. Trisomy 21 (Down syndrome)
d. Trisomy 16-18 (Edward syndrome)
e. Trisomy F (17-18)
f. Trisomy 13-15 (Patau syndrome)
g. Turner syndrome (XO, gonadal dysgenesis)
h. 9p syndrome
(4) Cockayne syndrome
(5) Congenital rubella syndrome
(6) Cretinism (juvenile hypothyroidism)
(7) Cystinosis
(8) Dental-ocular-cutaneous syndrome
(9) Diamond-Blackfan syndrome
(10) Ehlers-Danlos syndrome
(11) Familial histiocytic dermatoarthritis syndrome
(12) Fetal alcohol syndrome
(13) Gorlin-Goltz (multiple basal cell nevi) syndrome
(14) Hallermann-Streiff syndrome (oculomandibulofacial dyscephaly)
(15) Homocystinuria
(16) Kartagener syndrome (sinusitis-bronchiectasis-situs inversus)
(17) Kimmelstiel- Wilson syndrome
(18) Klinefelter syndrome
(19) Klippel-Trenaunay-Weber syndrome
(20) Krabbe syndrome
(21) Krause syndrome (congenital encephaloophthalmic dysplasia)
(22) Lowe (oculocerebrorenal) syndrome
(23) Marfan syndrome (arachnodactyly dystrophia mesodermalis
congenita)
(24) Meyer-Schwickerath-Weyers syndrome (oculodentodigital dysplasia)
(25) Miller (Wilms aniridia) syndrome
(26) MPS
a. Hurler syndrome (MPS IH)
b. Maroteaux-Lamy syndrome (MPS VI)
c. Morquio syndrome (MPS IV)
(27) Nieden (telangiectasia-cataract) syndrome
(28) Pierre Robin syndrome (micrognathia-glossoptosis) syndrome
(29) Prader-Willi syndrome (hypotonia, hypogonadism, obesity, and
mental retardation)
(30) Rubella syndrome
(31) Rubinstein-Taybi (broad thumb) syndrome
(32) Silverman (battered-child) syndrome
(33) Stickler syndrome (hereditary progressive arthroophthalmopathy)
(34) Treacher-Collins syndrome
(35) Ullrich syndrome (dyscraniopygophalangy)
(36) Waardenburg syndrome
(37) Wagner syndrome
(38) Weber-Christian disease
(39) Weil-Marchesani syndrome
(40) X-linked mental retardation (XLMR) syndrome
(41) Zellweger (cerebrohepatorenal) syndrome
*L. Diabetes
M. Epidemic dropsy (argemone oil poisoning)
N. Giant cell arteritis
*O. Graves disease
P. Hemorrhagic fever with renal syndrome (nephropathia epidemica)
*Q. Herpes simplex
*R. Herpes zoster
S. Histiocytosis X
T. JRA
U. Juvenile xanthogranuloma
V. Leukemia
W. Lymphoma
X. Medications or chemicals
Y. Metastatic carcinoma
Z. Metastatic melanoma
AA. Multiple myeloma
BB. Phakomatoses
(1) Nevus of Ota (oculodermal melanocytosis)
(2) Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
(3) von Hippel-Lindau disease
(4) von Recklinghausen neurofibromatosis
CC. Reiter syndrome
DD. Retinoblastoma
EE. Retrobulbar tumors
FE Sarcoidosis
GG. Sickling disorders
HH. Superior vena cava (superior mediastinal) syndrome
II. Syphilis
JJ. Systemic corticosteroids
KK. Vogt-Koyanagi-Harada syndrome
Evans LS. Increased intraocular pressure in severely burned patients. Am J Ophthalmol

1991; 111:56-58.
Grant WM. Toxicology of the eye, 4th ed. Springfield, IL: Charles C Thomas, 1993.
GLAUCOMA SUSPECT, ADULT
1. Advanced age
*2. Applanation reading 21 mm Hg or greater
3. Asymmetric intraocular pressures (IOPs)
4. Black race
5. Contusion-angle deformity glaucoma in the fellow eye
*6. Diabetes mellitus
7. Diurnal fluctuation in IOP of 10 mm Hg or greater
8. Endothelial dystrophy of the cornea
*9. Exfoliative syndrome (see p. 400)
10. Family history of glaucoma
* 11. Hemorrhage at optic disc margin
12. High myopia
13. IOP elevation following use of corticosteroids
14. Krukenberg spindle or dense trabecular pigment band
*15. Prominent cupping of optic disc
A. Asymmetry of cup-to-disc ratio
B. Cup-to-disc ratio 0.4
C. Cupping-to-disc margin
D. Vertical elongation of cup
16. Retinal detachment (see p. 487)
*17. Retinal vein occlusion (see p. 468-472)
18. Schiötz scale reading 4.0/5.5 or 6.25/7.5 or less
19. Thyrotropic exophthalmos
*20. Visual field changes suggestive of glaucoma
ELEVATED INTRAOCULAR PRESSURE MEASUREMENT WITH

NORMALAPPEARING
OPTIC DISC
1. Acromegaly
2. Anesthesia
A. Ketamine
B. Nitrous oxide with intravitreal gas
C. Succinylcholine
*3. Blepharospasm
4. Caffeine intake
5. Cardiopulmonary bypass surgery
*6. Dysthyroid ophthalmopathy
7. Elevation in hemoglobin concentration
8. Excessive water intake
9. High scleral rigidity and indentation (e.g., Schiötz) tonometry
10. Horizontal gaze position
11. Hyperthermia
12. Hyperthyroid
13. Marked emotional stress
14. Mechanical factors in checking IOP (e.g., by patient's hair interfering with
applanation tonometer arm)
15. Medications or chemicals
*A. Corticosteroids
B. Cycloplegics
C. Others
*16. Normal variation (ocular hypertension)
*17. Preglaucoma (IOP sufficiently elevated to cause damage to the optic nerve, but
damage is not yet visible ophthalmoscopically)
18. Reduced gravity
19. Tight collar, short neck, obesity
20. Tobacco smoking
*21. Tonometer in need of calibration
22. Valsalva maneuver
23. Voluntary widening of palpebral fissure
Munoz M, Capo H. Differential intraocular pressure in restrictive strabismus. Am J

Ophthalmol 1991; 112:352-353.
Shields MB. Textbook of glaucoma. Baltimore: Williams & Wilkins, 1992.

SECONDARY OPEN-ANGLE GLAUCOMA
1. Corneal endothelial disorders
A. Fuchs endothelial dystrophy
B. Posterior polymorphous dystrophy
2. Elevated episcleral venous pressure (see p. 235)
3. Iris disorders
A. Iridoschisis
*B. Pigmentary glaucoma
4. Lens disorders
A. Cataract
(1) Lens particle glaucoma
(2) Phacoanaphylaxis
(3) Phacolytic (lens protein) glaucoma
B. Displaced lens (see p. 317-318)
*C. Exfoliation syndrome
*A. Corticosteroids
B. Cycloplegic effect
C. Others
6. Ocular hemorrhage
7. Ocular inflammation
8. Ocular surgery
A. Alpha-chymotrypsin (enzyme glaucoma)
*B. Corticosteroid induced
C. Distortion of anterior chamber angle from limbal or keratoplasty sutures
D. Early postoperative elevation of IOP following cataract surgery (especially in
eyes with preexisting glaucoma)
E. Hemorrhage
(1) Degenerated ocular blood
a. Ghost cell glaucoma
b. Hemolytic glaucoma
c. Hemosideric glaucoma
*(2) Hyphema
(3) Internal wound neovascularization (late postoperative hyphema)
(4) Pseudophakia
a. Anterior chamber intraocular lens (IOL), including uveitis,
glaucoma, hyphema (UGH) syndrome
b. Iris-fixated IOL
c. Posterior chamber IOL (usually sulcus fixation)
(5) Retrobulbar hemorrhage (massive)
F. Inflammation
G. Intravitreal gas
H. Neodymium: yttrium-aluminum-aluminum-garnet (Nd:YAG) laser
capsulotomy
I. Pseudophakic pigmentary dispersion (e.g., with posterior chamber implant)
J. Retained lens cortex
*K. Retained viscoelastic
L. Silicone oil
M. Sudden closure of cyclodialysis cleft
N. Vitreous filling anterior chamber
9. Ocular trauma
A. Chemical bums (acid, alkali, other)
B. Contusion
(1) Angle recession
*(2) Hyphema
(3) Iritis
(4) Trabecular damage
C. Radiation damage
D. Retained intraocular foreign body (iron, copper)
E. Retrobulbar hemorrhage (massive)
10. Ocular tumors
11. Retinal, vitreous, and choroidal disorders
*A. Neovascular glaucoma-open-angle stage (see p. 324)
B. Retinitis pigmentosa
C. Rhegmatogenous retinal detachment (Schwartz syndrome)
12. Systemic, such as ocular amyloidosis
Nelson GA, et al. Ocular amyloidosis and secondary glaucoma. Ophthalmology

106:1363-1366.
UNILATERAL GLAUCOMA
1. Corneal endothelial disorders

A. Fuchs endothelial dystrophy with angle closure due to thickened peripheral
cornea
B. Iridocorneal endothelial (ICE) syndrome
(1) Chandler syndrome
(2) Iris-nevus (Cogan-Reese) syndrome
(3) Progressive iris atrophy
2. Elevated episcleral venous pressure (see p. 235)
3. Lens disorders
A. Cataract
(1) Lens-particle glaucoma
(2) Phacoanaphylaxis
(3) Phacolytic (lens protein) glaucoma
(4) Phacomorphic (intumescent lens) glaucoma
B. Displacement of the lens
(1) Buphthalmos
(2) Cataract (mature or hypennature)
(3) Exfoliation syndrome
(4) Intraocular tumor
(5) PHPV
(6) Sturge- Weber syndrome (encephalotrigeminal angiomatosis)
(7) Trauma
(8) Uveitis
C. Exfoliation syndrome (see p. 4(0)
B. Chemical burns
*C. Corticosteroids (topical or periocular)
D. Cycloplegics (angle closure or open angle)
E. Nitrous oxide inhalation with intraocular gas
F. Urokinase (intraocular)
G. Others
5. Ocular hemorrhage
6. Ocular inflammation
*7. Ocular surgery
8. Ocular trauma
9. Ocular tumors
10. Retinal, vitreous, and choroidal disorders
A. Angle closure
(1) Acute choroidal hemorrhage
*(2) CRVO
(3) Ciliochoroidal effusion
a. Arteriovenous malformations
b. Inflammatory conditions
c. Nanophthalmos
d. Surgery
e. Trauma
f. Tumors
g. Uveal effusion syndrome
(4) Hemorrhagic retinal and choroidal detachment
(5) PHPV
*(6) Postoperative panretinal photocoagulation
(7) Postoperative scleral buckle
(8) Retinal dysplasia
(9) Retinopathy of prematurity (retrolental fibroplasia)
*B. Neovascular glaucoma (see p. 324)
*(1) Diabetic retinopathy
(2) Extraocular vascular disorders
a. Carotid-cavernous fistula
b. Carotid occlusive disease
c. Giant cell arteritis
(3) Ocular disorders-miscellaneous
*a. Chronic glaucoma
b. Endophthalmitis
c. Intraocular malignancy
d. Iris melanoma
e. PHPV
f. Photoradiation or helium ion irradiation for uveal melanoma
g. Pseudophakia
h. Sympathetic ophthalmia
*i. Uveitis (chronic)
(4) Retinal disorders-miscellaneous
a. Coats disease
b. Eales disease
c. Optic-nerve glioma with venous stasis
d. Retinal detachment (usually chronic)
e. Retinal vascular occlusive disorders
1. Retinal artery occlusion central or branch
*2. Retinal vein occlusion central or branch
f. Retinoblastoma
g. Retinopathy of prematurity (retrolental fibroplasia)
h. Retinoschisis
i. Sickle cell retinopathy
C. Open-angle glaucoma associated with rhegmatogenous retinal detachment
(Schwartz syndrome)
Shields MB. Textbook of glaucoma, 3rd ed. Baltimore: Williams & Wilkins. 1992.
GLAUCOMA ASSOCIATED WITH DISPLACED LENS
1. Alport syndrome
2. Aniridia
3. Axenfeld-Rieger syndrome
4. Buphthalmos
*5. Cataract (mature or hypermature)
6. Cornea plana
8. Ectopia lentis et pupillae
9. Ehlers-Danlos syndrome
*10. Exfoliation syndrome (see p. 400)
11. High myopia
12. Homocystinuria
13. Hyperlysinemia
15. Isolated microspherophakia
16. Klinefelter syndrome
17. Lowe (oculocerebrorenal) syndrome
*18. Marfan syndrome
19. Megalocornea
20. Oculodental syndrome
21. Refsum syndrome
23. Scleroderma
24. Simple ectopia lentis
25. Stickler syndrome
26. Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
27. Sulfite oxidase deficiency
28. Syphilis
*29. Trauma
30. Treacher-Collins syndrome (mandibulofacial dysostosis)
31. Uveitis
32. Weill-Marchesani syndrome
Epstein DL. Chandler and Grant's glaucoma, 3rd ed. Philadelphia: Lea & Febiger, 1986.
GLAUCOMA AND ELEVATED EPISCLERAL VENOUS PRESSURE
1. Arteriovenous fistulas
A. Carotid-cavernous sinus fistulas
(1) Spontaneous
(2) Traumatic
B. Orbital-meningeal shunts
C. Orbital varices
*D. Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
2. Idiopathic elevation of episcleral venous pressure
*A. Familial
B. Sporadic
3. Venous obstruction
A. Cavernous sinus thrombosis
*B. Congestive heart failure
C. Episcleral
(1) Chemical bums (acid, alkali, and others)
(2) Radiation
D. Jugular venous obstruction
E. Ocular amyloidosis
F. Orbital
*(1) Dysthyroid
(2) Orbital vein thrombosis
(3) Phlebitis
(4) Pseudotumor
(5) Retrobulbar tumor
G. Pulmonary venous obstruction
H. Superior vena cava (superior mediastinal) syndrome
Weinreb RN, et al. Glaucoma secondary to elevated episcleral venous pressure. In: Ritch
R, et aI. The glaucomas. St. Louis: CV Mosby, 1989.
GLAUCOMA ASSOCIATED WITH SHALLOW ANTERIOR CHAMBER
*1. Primary-angle closure

A. Plateau iris syndrome
*B. Relative pupillary block (most common)
2. Secondary-angle closure
*A. CRVO (central retinal vein occlusion)
B. Choroidal hemorrhage (acute)
C. Ciliochoroidal effusion
(1) AIDS
(2) Arteriovenous malformations
*(3) Inflammation
(4) Nanophthalmos
(5) Trauma
(6) Tumor
(7) Uveal effusion syndrome
D. Cystinosis
E. Drug-induced acute transitory myopia (diuretics, sulfonamides, and others)
F. Elevated episcleral venous pressure associated with arteriovenous fistula
G. Fuchs endothelial dystrophy-with peripheral corneal thickening
H. Hemorrhagic retinal and choroidal detachment
I. Hyperglycemia (acute)
J. Inflammation
(1) Episcleritis
(2) Iridocyclitis with posterior synechiae and iris bombe
(3) Posterior scleritis
K. Intraocular tumor (posterior segment melanoma, metastatic carcinoma,
retinoblastoma, medulloepithelioma, and others)
L. Lens dislocation (see p. 401-404)
M. Luetic interstitial keratitis
N. Malignant (ciliary block) glaucoma
O. Maroteaux-Lamy syndrome (MPS VI)
P. Multiple cysts of the iris and ciliary body
Q. Nanophthalmos
R. Pars plana vitrectomy
S. PHPV
*T. Phakic or aphakic pupillary block
U. Phakomorphic (intumescent lens) glaucoma
V. Postoperative panretinal photocoagulation
W. Postoperative scleral buckle
X. Pupil dilatation, including topical dilatation and systemic decongestants,
bronchodilators, gastroenterologic and genitourinary disorders.
Y. Retinal dysplasia
Z. Retinopathy of prematurity (retrolental fibroplasia)
2000.
Mungan N, et al. Ultrasound biomicroscopy of the eye in cystinosis. Arch Ophthalmol

2000; 118:1329-1333.
Rho DS. Acute angle-closure glaucoma after albuterol nebulizer treatment. Am J

Ophthalmol 2000; 130:123-124.
GLAUCOMA IN APHAKIA OR PSEUDOPHAKIA
1. Alpha-chymotrypsin (enzyme glaucoma)

2. Ciliary-block (malignant) glaucoma
*3. Corticosteroid induced
4. Degenerated intraocular blood
A. Ghost cell glaucoma
B. Hemolytic glaucoma
C. Hemosideric glaucoma
5. Distortion of the anterior chamber angle by limbal sutures
*6. Early postoperative pressure elevation (especially in eyes with preexisting glaucoma)
7. Epithelial downgrowth
8. Fibrous proliferation
*9. Following Nd:YAG capsulotomy
10. Hyphema
A. Internal wound neovascularization (late postoperative hyphema)
B. Pseudophakia
(1) Anterior chamber IOL (including the UGH syndrome)
(2) Iris-fixated IOL
(3) Posterior-chamber IOL (usually sulcus fixation)
11. Inflammation
12. Peripheral anterior synechiae
13. Primary open-angle glaucoma
14. Pseudophakic pigmentary dispersion
*15. Pupillary block
*16. Retained lens cortex
*17. Retained viscoelastic
18. Vitreous filling the anterior chamber
19. Vitreous hemorrhage (massive)
Duane TD, Jaeger EA. Clinical Ophthalmology. New York: Harper & Row. 1994.
Tessler DR, et al. Persistently raised intraocular pressure following extracapsular cataract
extraction. Br J Ophthalmol 1990; 74:272-274.
MEDICATIONS AND CHEMICALS THAT MAY CAUSE ELEVATED

INTRAOCULAR PRESSURE
1. Anesthetic agents
A. Ketamine
B. Nitrous oxide (inhalation, especially in eyes with retinovitreal surgery and
intraocular gas)
2. Anticholinergics/parasympatholytics
A. Antidepressants
(1) Amitriptyline (Elavil)
(2) Imipramine (Tofranil)
(3) Nortriptyline (Pamelor)
(4) Protriptyline (Vivactil)
(5) Trimipramine (Surmontil)
B. Antihistamines
(1) Anazolene (Vasocon-A)
(2) Brompheniramine (Dimetane)
(3) Cyclizine (Marezine)
(4) Cyproheptadine (Periactin)
(5) Diphenhydramine (Benadryl)
(6) Orphenadrine (Norgesic)
(7) Tripelennamine (Pyribenzamine)
C. Antiparkinson medications
(1) Biperiden (Akineton)
(2) Cycrimine (Pagitane)
(3) Trihexyphenidyl hydrochloride (Artane)
D. Antispasmodic agents
(1) Dicyclomine (Bentyl)
(2) Diphemanil methylsulfate (Prantal)
(3) Hexocyclium methylsulfate (Tral)
(4) Hyoscyamine (Donnatal, Donnagel)
(5) Mepenzolate (Cantil)
(6) Methscopolamine bromide (Pamine)
(7) Oxyphenonium bromide (Antrenyl)
(8) Propantheline bromide (Pro-Banthine)
(9) Tridihexethyl chloride (Pathilon)
*E. Cycloplegics
(1) Atropine
(2) Cyclopentolate (Cyclogyl)
(3) Homatropine
(4) Tropicamide (Mydriacyl)
(5) Scopolamine (Hyoscine)
F. Miscellaneous
(1) Atropine (systemic)
(2) Glycopyrrolate (Robinul)
G. Phenothiazine
(1) Doxepin (Sinequan)
(2) Haloperidol (Haldol)
(3) Prochlorperazine (Compazine)
(4) Promethazine (Phenergan)
(5) Triflupromazine (Vesprin)
H. Poisoning
(1) Belladonna
(2) Jimson weed
3. Argemone oil (epidemic dropsy)
4. Caffeine
5. Carbon dioxide inhalation
6. Carmustine injection
7. Chemical bums
A. Acid
(1) Chromic acid
(2) Hydrochloric (muriatic) acid
(3) Sulfuric (battery) acid
B. Alkali
(1) Ammonium hydroxide (ammonia)
(2) Calcium hydroxide (lime)
(3) Sodium hydroxide (lye)
C. Dibent [b.f][l,4] oxazepine (CR tear gas)
D. Formaldehyde gas (in aqueous solution formalin)
8. CNS stimulants/anorexics
A. Dextroamphetamine
B. Methamphetamine
C. Phenmetrazine (Preludin)
D. Phentermine (Ionamin)
*9. Corticosteroids
*A. Ocular (topical)
(1) Dexamethasone (Decadron, Maxidex)
(2) Fluorometholone (FML, Flarex)
(3) Prednisolone acetate (Pred Forte)
(4) Prednisolone sodium phosphate (Inflamase)
*B. Subconjunctival depot injection
(1) Methylprednisolone acetate
(2) Triamcinolone
C. Systemic
(1) Betamethasone (Celestone)
(2) Cortisone acetate
(3) Dexamethasone (Decadron)
(4) Hydrocortisone (Cortef, Solu-Cortef)
(5) Methylprednisolone (Medrol)
(6) Paramethasone (Haldrone)
(7) Prednisolone
(8) Prednisone (Deltasone)
(9) Triamcinolone (Aristocort)
10. Idiopathic lens swelling
A. Acetylsalicylic acid (aspirin)
B. Sulfanilamide
C. Others
11. Intraocular injection
B. Urokinase
*C. Viscoelastic (Healon, others)
12. Methylphenidate (Ritalin)
13. Miotics
A. Carbachol
B. Demecarium (Humorsol)
C. Echothiophate (phospholine iodide)
D. Pilocarpine
14. Succinylcholine (Anectine)
15. Sympathomimetics
A. Ephedrine
B. Mydriatics
(1) Dipivalyl epinephrine (Propine)
(2) Epinephrine (many products)
(3) Hydroxyamphetamine (paredrine)
(4) Phenylephrine (Neo-Synephrine)
C. Naphazoline (Naphcon)
D. Pheniramine maleate (Naphcon-A)
E. Phenylephrine (Neo-Synephrine)
F. Tetrahydrozoline (Visine)
16. Testosterone
17. Vasodilators
A. Elevation of IOP following subconjunctival injection
(1) Bamethan (Bupatol)
(2) Isoxsuprine (Vasodilan)
(3) Tolazoline (Priscoline)
(4) Triaziquone (Trenimon)
B. Amyl nitrite (Vaporole)
18. Water (excessive intake)
May be potentiated by monoamine oxidase inhibitors such as phenelzine,
pargyline, or tranylcypromine.
PRIMARY LOW-TENSION GLAUCOMA
1. Nonglaucomatous optic nerve disorders resembling glaucomatous damage

A. Developmental abnormalities
(1) Colobomas of the optic-nerve head, including optic pits
*(2) Large physiologic cups
(3) Tilted discs
B. Nonglaucomatous causes of acquired cupping
(1) Compressive lesions
a. Aneurysm
b. Chiasmic arachnoiditis
c. Cyst
d. Tumor
*(2) Ischemic optic neuropathy (especially arteritic)
*C. Nonglaucomatous causes of nerve fiber bundle defects on visual field testing
(1) Chorioretinal lesions
a. Chorioretinitis
b. Retinal vascular occlusions
c. Tumors
(2) Optic-nerve head lesions
a. Colobomas
b. Drusen
c. Other
(3) Posterior lesion of the visual pathway
a. Meningioma
b. Pituitary tumor
c. Pseudotumor
d. Other
2. Undetected high-pressure glaucoma
A. Corneal edema giving false low measurement of IOP with applanation (e.g.,
Goldman or Perkins) tonometry
B. Intermittent elevation of IOP causing damage (IOP normal at time of
examination)
(1) Glaucomatocyclitic crisis (Posner-Schlossman syndrome)
(2) Intermittent angle closure
(3) Others
C. Low scleral rigidity giving false low measurement of IOP with indentation
(e.g., Schiötz) tonometry
*D. Prior elevation in pressure resulting in optic nerve damage
(1) Burned-out open-angle glaucoma
(2) Corticosteroids
(3) Pigmentary glaucoma
(4) Trauma
(5) Uveitis
E. Wide diurnal variation (multiple measurements at different times of day
required to rule-out high-pressure glaucoma)
Werner EB. Low-tension glaucoma. In: Ritch R, et al. The glaucomas. St. Louis: CV
Mosby, 1989.
NEOVASCULAR GLAUCOMA
*1. Diabetic retinopathy

2. Extraocular vascular disorders
A. Aortic arch syndrome
*B. Carotid artery occlusive disease
C. Carotid-cavernous fistula
D. Giant cell arteritis
3. Ocular disorders-miscellaneous
A. Chronic glaucoma
B. Endophthalmitis
C. Iris melanoma
D. PHPV
E. Pseudophakia
F. Sympathetic ophthalmia
*G. Uveitis
4. Retinal disorders-miscellaneous
A. Choroidal melanoma
B. Coats exudative retinopathy
C. Eales disease
D. Metastatic carcinoma
E. Norrie disease
F. Optic nerve glioma with subsequent venous stasis retinopathy
G. Photoradiation or helium ion irradiation for uveal melanoma
*H. Retinal detachment (usually chronic)
I. Retinal vascular occlusive disorders
(1) Branch retinal artery occlusion.
*(2) Branch retinal vein occlusion
*(3) Central retinal artery occlusion
*(4) Central retinal vein occlusion
J. Retinoblastoma
K. Retinopathy of prematurity (retrolental fibroplasia)
L. Retinoschisis
M. Sickle cell retinopathy
N. Stickler syndrome (inherited vitreoretinal degeneration)
O. Syphilitic retinal vasculitis
HYPOTONY
*1. Essential hypotension

2. Secondary hypotony
A. Cartilaginous-arthritic-ophthalmic deafness
B. Ciliochoroidal detachment
(1) Chorioretinal inflammation
(2) Ocular neoplasm
*(3) Trauma, including ocular surgery
*C. Cyclitis
*D. Cyclodialysis
E. Decreased IOP from medications and chemicals
(1) Alcuronium
(2) Aminophylline (intravenous)
*(3) Carbonic anhydrase inhibitors (e.g., acetazolamide, methazolamide,
ethoxzolamide)
(4) Cardiac glycosides (digitoxin, digoxin, lanatoside-C, ouabain)
(5) Dibenamine
(6) Dihydroergotoxine (Hydergine)
(7) HPMPC (cidofovir)
(8) Hyperosmotics (urea, glycerin, mannitol, ascorbic acid, glycerol,
ethanol, trometamol)
(9) Isosorbide
(10) Pargyline (Eutonyl)
(11) Phentolamine (Regitine)
(12) Propranolol (Inderal)
(13) Thiopental (Pentothal)
*F. Deep anesthesia
G. Deep coma and severe cerebral disease
*H. Dehydration-severe (e.g., cholera, dysentery, diabetic coma)
*1. Diabetic coma
*J. Glaucoma medications (beta-blockers, sympathomimetics, miotics, carbonic
anhydrase inhibitors)
K. Hilding syndrome
L. Homer syndrome
M. Hyperosmolarity
N. Intestinal perforation or obstruction
O. Intraocular lens mal position
P. Irradiation
Q. Morquio-Brailsford syndrome (MPS IV)
R. Myotonic dystrophy
S. Ocular ischemia
*T. Ocular trauma with or without visible ciliary body injury
*U. Perforating ocular trauma
*V. Phthisis
W. Postencephalitic syndrome
*X. Postoperative surgical procedures especially for glaucoma
Y. Raeder syndrome
*Z. Retinal detachment
*AA. Systemic hypotension-severe (circulatory collapse, medications)
BB. Uremic coma
*CC. Wound leak
Scott RA, Pavesio C. Ocular side-effects from systemic HPMPC (Cidofovir) for a non-
ocular cytomegalovirus infection. Am J Ophthalmol 2000; 130:126-127.
Shields MB. Textbook of glaucoma. 3rd ed. Baltimore: Williams & Wilkins, 1992.
GLAUCOMA ASSOCIATED WITH UVEITIS
1. Arthritis
3. Ankylosing spondylitis, Reiter syndrome, psoriatic arthritis
6. Fuchs heterochromic uveitis
7. Posner-Schlossman syndrome (glaucomatocyclitic crisis)
8. Herpes simplex
9. Herpes zoster
10. Hansen disease
11. Rubella
12. Mumps and other viral infections
13. Gnathostomiasis
14. Traumatic uveitis
15. Phacoanaphylactic glaucoma
16. Phacolytic glaucoma
17. Pseudophakic-inflammatory glaucoma
18. Intermediate uveitis (pars planitis)
19. Posterior uveitis
a. Ocular toxoplasmosis
b. Acute retinal necrosis
c. AIDS
d. Other posterior uveitides
20. Panuveitis
a. Sarcoidosis
b. Behçet syndrome
c. Sympathetic ophthalmia
d. Vogt-Koyanagi-Harada syndrome
e. Congenital syphilis
f. Acquired syphilis
g. Tuberculosis
h. Onchocerciasis
21. Masquerade syndromes
a. Intraocular neoplasia (uveal malignant melanoma, intraocular
lymphoma, and others)
b. Retinal detachment
22. Open-angle glaucoma
23. Angle-closure glaucoma
24. Combined-mechanism glaucoma
Moorthy RS, et al. Glaucoma associated with uveitis. Surv Ophthal 1997; 5:361-394.
GLAUCOMA ASSOCIATED WITH INTRAOCULAR TUMORS
1. Iris
A. Nevus
B. Melanocytoma
C. Iris pigment epithelium adenoma
D. Malignant melanoma
E. Metastatic
2. Ciliary body
A. Medulloepithelioma
B. Melanocytoma
C. Malignant melanoma
D. Metastatic
3. Choroid
A. Malignant melanoma
B. Metastatic
4. Optic nerve
A. Melanocytoma
B. Metastatic
5. Retina-retinoblastoma
6. Metastatic
A. Carcinoma
B. Cutaneous melanoma
C. Breast
D. Lung
E. Kidneys
F. Testicles
G. Prostate
H. Pancreas
I. Colon
J. Gastrointestinal
7. Others
A. Leukemia
B. Lymphoma
C. Phakomatoses as Sturge-Weber or neurofibromatosis
D. Multiple myelomas
E. Juvenile xanthogranuloma
Shields CL, et al. Prevalence and mechanisms of secondary intraocular pressure elevation
in eyes with intraocular tumors. Ophthalmolology 1987; 94:839-846.
Sullivan L, et al. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr

Ophthalmol Strabismus 1992; 29:349-356.
10
Anterior Chamber
CONTENTS
Hypopyon 329
Hyphema 331
Spontaneous hyphema 332
Spontaneous hyphema in infants 333
Plasmoid aqueous 338
Cholesterolosis of the anterior chamber 338
Gas bubbles in the anterior chamber 338
Pigmentation of trabecular meshwork 338
Pigment liberation into the anterior chamber with dilatation of pupil 339
Grading of anterior chamber angle width 339
Blood in Schlemm canal 339
Deep anterior chamber angle 340
Narrow anterior chamber angle 340
Irregular depth of the anterior chamber 341
Peripheral anterior synechiae 341
Neovascularization of anterior chamber angle 342
Iris processes 343
White mass in anterior chamber 343
HYPOPYON (PUS IN ANTERIOR CHAMBER)
1. Hypopyon ulcer-corneal ulcer with pus in the anterior chamber

A. Acanthamoeba
B. Acquired immunodeficiency syndrome (AIDS)
C. Aspergillus species
D. Candida albicans
E. Chemical injury
F. Diplococcus pneumoniae
G. Escherichia coli
H. Fusariam species
I. Herpes simplex
J. Herpes zoster
K. Measles
L. Moraxella
M. Neisseria gonorrhoeae
N. Proteus vulgaris
*O. Pseudomonas aeruginosa
P. Serratia species
Q. Smallpox
R. Spitting-cobra venom
S. Staphylococcus
*T. Streptococcus
2. Severe acute iridocyclitis
3. Repeated corneal transplantation of human amniotic membrane
4. Necrosis of intraocular tumors or metastasis
5. Retained intraocular foreign bodies, including toxic lens syndrome
6. Endophthalmitis-at time of surgical treatment, accidental trauma, in drug users, or
spontaneous occurrence (see p. 223)
A. Acanthamoebae
B. Actinomycosis
C. Amebiasis
D. Aspergillosis species
E. Bacterial including bacillus cereus
F. Behçet syndrome
G. C. albicans
H. Coccidioidomycosis
I. Coenurosis
J. Cysticercosis
K. Fusarium species
L. Hydatid cyst
M. Influenza
N. Listeria monocytogenes
O. Lockjaw (Clostridium tetani)
P. Metastatic bacterial endophthalmitis
Q. Moraxella species
R. Mucor species
S. Mycobacterium avium
*T. Pseudomonas species
U. Relapsing fever
V. Serratia marcescens
W. Saprophytic fungi
*X. Staphylococcus
*Y. Streptoccus
Z. Sterile hypopyon
(1) Behçet syndrome (oculobuccogenital syndrome)
(2) Endotoxin contamination of ultrasonic bath
(3) Following cyanoacrylate sealing of a corneal perforation
(4) Following refractive surgery
(5) Histiocytosis X (Hand-Schüller-Christian syndrome)
(6) Intraocular lens or instrument polishing compounds or sterilization
techniques
*(7) Juvenile rheumatoid arthritis
(8) Laser iridotomy
(9) Leukemia
*(10) Reaction to lens protein
(11) Rough intraocular lens edges
(12) von Bechterev-Strumpel syndrome (rheumatoid spondylitis)
AA. Stevens-Johnson syndrome (dermatostomatitis)
BB. Tight contact lens or contact lens overwear syndrome
CC. Tuberculosis
DD. Weil disease (leptospirosis)
EE. Yersiniosis
benoxinate ferrous succinate polysaccharide-iron
butacaine ferrous sulfate complex
cocaine iodide and iodine proparacaine
colchicine (?) solutions and radioactive iodides
dibucaine compounds rifabutin
dyclonine iron dextran tetracaine urokinase
ferrocholinate iron sorbitex urokinase
ferrous fumarate phenacaine
ferrous gluconate piperocaine
7. Vitreous "fluff-ball"
8. Following refractive surgery
9. Pseudohypopyon
A. Ghost cell glaucoma with khaki-colored cells
B. Accidental intraocular steroid injection
10. Acute angle-closure glaucoma
11. Non-Hodgkin lymphoma
12. Pars plana vitrectomy and silicone oil injection
Gabler B, Lohmann CP. Hypopyon after repeated transplantation of human amniotic

membrane onto the corneal surface. Ophthalmology 2000; 107:1344-1346.
Recchia FM, et al. Endophthalmitis after pediatric strabismus surgery. Arch Ophthalmol
2000; 118:939-944.
Saran BR, et al. Hypopyon uveitis in patients with acquired immunodeficiency syndrome
treated for systemic Mycobacterium avium complex infection with rifabutin. Arch
Ophthalmol 1994; 112; 1159-1161.
HYPHEMA (BLEEDING INTO THE ANTERIOR CHAMBER)

1. Trauma
A. Following laser iridectomy or strabismus surgery in aphakia
B. Honan balloon use in Fuchs heterochromic iridocyclitis
*C. Tear of ciliary body-post contusion deformity of anterior chamber
*D. To ciliary body, such as cyclodialysis
E. To iris, such as in iridodialysis or intraocular lens irritation
F. After airbag inflation
G. Metallic intraocular foreign body during magnetic resonance imaging
2. Overdistention of vessels
A. Obstruction of central retinal vein
B. Sudden lowering of high intraocular pressure
3. Fragility of vessel walls
A. Acute gonorrheal iridocyclitis
B. Acute herpes iridocyclitis
C. Acute rheumatoid iridocyclitis
D. Ankylosing spondylitis
4. Blood abnormality
A. Anemias
B. Association with use of aspirin
C. Hemophilia
D. Leukemia
E. Purpura
F. Sickle cell disease
5. Metabolic disease
A. Diabetes mellitus (Willis disease)
B. Scurvy (avitaminosis C)
6. Neovascularization of iris (see rubeosis iridis, p. 366-367)
7. Vascularized tumors of iris (see pigmented and nonpigmented iris lesions, p. 374-375)
A. Angioma
B. Iris vascular tufts
*C. Juvenile xanthogranuloma (JXG)
D. Lymphosarcoma
E. Retinoblastoma
8. Wound vascularization following cataract extraction
9. Persistent pupillary membrane hemorrhage
Dahlmann AH, et al. Spontaneous hyphema secondary to iris vascular tufts. Arch
Ophthalmol 2001; 119: 1728-1729.
Gottsch JD. Hyphema: diagnosis and management. Retina 1990; 10:65-72.
Keszel VA, Helveston EM. Hyphema as a complication of strabismus: surgery in an

aphakic eye. Arch Ophthalmol 1986; 104:637-638.
Ta CN, Bowman RW. Hyphema caused by a metallic intraocular foreign body during
magnetic resonance imaging. Am J Ophthalmol 2000; 129:533-534.
SPONTANEOUS HVPHEMA
1. Delayed following glaucoma surgery

2. Diseases of blood or blood vessels
A. Hemophilia
B. Leukemia
C. Malignant lymphoma
D. Purpura
E. Scurvy
3. Fibrovascular membranes in retrolenticular or zonular area
A. Persistent primary vitreous
B. Retinoschisis
C. Retinopathy of prematurity
4. Systemic hypertension
5. Hydrophthalmos
6. Iatrogenic
7. Intraocular neoplasms
*8. JXG-yellow nodules of skin and iris
9. Malignant exophthalmos
10. Microbial keratitis, especially Moraxella
11. Occult trauma or trauma with late effect
*12. Rubeosis iridis
13. Severe iritis with or without
A. Behçet disease (dermatostomatoophthalmic syndrome)
B. Diabetes mellitus (Willis disease)
C. Gonococcal infection
D. Herpes zoster or herpes simplex
14. Use of warfarin, heparin, aspirin, or alcohol
15. Vascular anomalies of iris
16. Wound vascularization following cataract extraction
Koehler MP, Shelton DB. Spontaneous hyphema resulting from warfarin. Ann
Ophthalmol 1983; 15:858-859.
Mason GI, et al. Bilateral spontaneous hyphema arising from iridic microhemangiomas.
Ann Ophthalmol 1979; 11:87.
Ormerod LD, Egan KM. Spontaneous hyphaema and corneal haemorrhages as

complications of microbial keratitis. Br J Ophthalmol 1987; 71:933.
Pandolfi M. Hemorrhages in ophthalmology. New York: Thieme-Stratton, 1979.
SPONTANEOUS HYPHEMA IN INFANTS
1. Acute rheumatoid iridocyclitis

2. Blood dyscrasias, such as anemia, leukemia, and disseminated intravascular
coagulation
3. Iritis
*4. JXG
5. Perinatal asphyxia
7. Retinoblastoma
8. Retinoschisis
*10. Trauma without history (consider child abuse)
Appelboom T, Durso F. Retinoblastoma presenting as a total hyphema. Ann Ophthalmol

1985; 17:508-510.
Harley RD, et al. Juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus 1982;

19:33-39.
Ortiz JM, et al. Disseminated intravascular coagulation in infancy and in the neonate.
Arch Ophthalmol 1982; 100:1413-1415.
Extracted Table Spontaneous Hyphema
Extracted Table Spontaneous Hyphema in Infants
PLASMOID AQUEOUS (AQUEOUS WITH A HIGH PROTEIN CONTENT)
1. Rheumatoid arthritis
2. Serum sickness
3. Infection with gonococcus
4. Following paracentesis or intraocular operation, such as cataract extraction
5. Severe corneal ulceration
6. Trauma
1991.
CHOLESTEROLOSIS OF THE ANTERIOR CHAMBER

In this condition, cholesterol crystals develop in the anterior chamber; usually in a
blind eye following trauma, but can be associated with hyphema or secondary glaucoma.
It is also associated with the following:
*1. Chronic uveitis

2. Eales disease (periphlebitis)
3. Lens subluxation
4. Mature or hypermature cataract
5. Microphthalmia
*6. Phthisis bulbi
7. Retinal detachment
8. Traumatic cataract
9. Vascular disorders
10. Vitreous hemorrhage
Mishra RK, et al. Cholesterol crystals in Eales disease. Indian J Ophthalmol 1980; 28:67-
68.
Wand M, Garn RA. Cholesterolosis of the anterior chamber. Am J Ophthalmol 1974;

78:143-144.
GAS BUBBLES IN THE ANTERIOR CHAMBER
2. E. coli
3. Yttrium-aluminum-garnet (YAG) laser treatment to the anterior segment
4. Postoperative intraocular surgery
Frantz JF, et al. Acute endogenous panophthalmitis caused by clostridium perfringens.

Am J Ophthalmol 1974; 78: 295-303.
Obertymski H, Dyson C. Clostridium peifringens panophthalmitis. Can J Ophthalmol

1974; 9:258-259.
PIGMENTATION OF TRABECULAR MESHWORK
1. In elderly individuals-inferior nasal or faint band circumferential

*2. Pseudoexfoliation of lens with or without glaucoma-unilateral or bilateral
*3. Pigmentary glaucoma
*4. Krukenberg spindle without glaucoma
*5. Malignant melanoma-one eye
6. Cyst of pigment layer of iris-unilateral
7. Previous intraocular operation, inflammation, or hyphema-scattered, mostly in lower
angle
8. Nevus-dense, isolated patch
9. Open-angle glaucoma-patchy band, whole circumference
10. Following gamma irradiation for malignancy of nasal sinus
Roth M, Simmons RJ. Glaucoma associated with precipitates on the trabecular
meshwork. Ophthalmology 1982; 86:1614.
PIGMENT LIBERATION INTO THE ANTERIOR CHAMBER WITH

DILATATION OF PUPIL
2. Exercise
3. Hurler disease (mucopolysaccharidoses IH)
4. Low-tension glaucoma with pigment dispersion
Ritch R. Nonprogressive low tension glaucoma with pigmentary dispersion. Am J

Ophthalmol 1982; 94:190-196.
GRADING OF ANTERIOR CHAMBER ANGLE WIDTH (USUALLY

DETERMINED BY GONIOSCOPY)
1. Grade 0: No angle structures visible-narrow angle, complete or partial closure (angle

closure)
2. Grade 1: Unable to see posterior one half of trabecular meshwork-extremely narrow
angle (probably capable of angle closure)
3. Grade 2: Part of Schlemm canal is visible-moderately narrow angle (may be capable of
angle closure)
4. Grade 3: Posterior portion of Schlemm canal is visible-moderately open angle
(incapable of angle closure)
5. Grade 4: Ciliary body is visible-open angle (incapable of angle closure)
Shields MB. Textbook of glaucoma, 2nd ed. Baltimore: Williams & Wilkins, 1986.
BLOOD IN SCHLEMM CANAL (REVERSAL OF NORMAL PRESSURE

GRADIENT)
*1. Artifact of goniolens flange occluding the episcleral veins in one or more quadrants
2. High episcleral venous pressure
*A. Carotid-cavernous sinus fistula (Red-eyed shunt syndrome)
*B. Dural-cavernous fistula
C. Mediastinal tumors
D. Orbital arteriovenous fistula
E. Sturge-Weber syndrome (meningocutaneous syndrome)
F. Superior vena cava obstruction (superior vena cava syndrome)
G. Tetralogy of Fallot
3. Low intraocular pressure
A. Following trabeculectomy
B. Hypotony (see p. 325)
C. Intraocular inflammation
4. Normal eye
Namba H. Blood reflux into anterior chamber after trabeculectomy. Jpn J Ophthalmol
1983; 27:616-625.
Phelps CD, et al. Arterial anastomosis with Schlemm's canal. Trans Am Ophthalmol Soc
1985; 83:304-315.
Phelps CD, et al. The diagnosis and prognosis of atypical carotid cavernous fistula. Am J
Ophthalmol 1982; 93: 423-436.
DEEP ANTERIOR CHAMBER ANGLE
1. Normal variation
2. Aphakia
3. Myopia
4. Megalocornea or conical cornea including keratoconus (see p. 288)
6. Posterior dislocation of the lens (see p. 401-404)
7. Recession of anterior chamber angle
1991.
Shields MB. Textbook of glaucoma, 2nd ed. Baltimore: Williams & Wilkins, 1986.
NARROW ANTERIOR CHAMBER ANGLE (MAY BE CAPABLE OF ANGLE

CLOSURE GLAUCOMA)
1. Normal variation
*2. Predisposition to angle closure
3. Anterior dislocation of the lens
4. Hyperopia
5. Spherophakia and microcornea
6. Postoperative intraocular operation with leaking wound (see hypotony, p. 325)
7. Choroidal detachment (see p. 532-535)
*8. Pupillary block
9. Loss of aqueous from perforating ulcer, corneal wound, or staphyloma (see hypotony,
p.325)
10. Intumescent senile cataract
11. Traumatic cataract that fluffs up
12. Primary hyperplastic primary vitreous (PHPV)
13. Peripheral anterior synechiae (see p. 341)
*14. Posterior entrapment of aqueous humor (malignant glaucoma or ciliary-block
glaucoma)
acetazolamide neostigmine sulfamethizole
acetylcholine physostigmine sulfamethoxazole
alpha-chymotrypsin pilocarpine sulfamethoxypyridazine
demecarium sulfacetamide sulfanilamide
dichlorphenamide sulfachlorpyridazine sulfaphenazole
echothiophate sulfadiazine sulfapyridine
edrophonium sulfadimethoxine sulfasalazine
ethoxzolamide sulfamerazine sulfathiazole
isoflurophate sulfameter sulfisoxazole
methazolamide sulfamethazine
*16. Plateau iris

17. Diffuse ciliary body or iris tumor
1991.
Taylor BC, Winslow RL. Pseudophakic flat anterior chamber following retinal
detachment repair. Ophthalmology 1981; 88:935.
IRREGULAR DEPTH OF THE ANTERIOR CHAMBER
1. Partial dislocation of lens

2. Tumor of iris or ciliary body
3. Peripheral anterior synechiae on one side of the chamber (see p. 341)
4. Iris bombe or pupillary block
5. Ruptured lens capsule with swelling on one side
6. Anatomic narrowing superiorly
7. Subacute angle-closure glaucoma
8. Cyclodialysis and traumatic recession of chamber angle
1991.
PERIPHERAL ANTERIOR SYNECHIAE (ADHESION OF IRIS TISSUE

ACROSS ANTERIOR CHAMBER STRUCTURES IN VARIABLE AMOUNTS
NOTED WITH GONIOSCOPY)
1. Bridge corneoscleral trabecular meshwork to Schwalbe line or anterior to Schwalbe

line (uncommon)
A. Anterior chamber cleavage syndrome
(1) Axenfeld syndrome (posterior embryotoxon)
(2) Congenital central anterior synechiae
(3) Following intraocular lens implantation
(4) Reiger syndrome (dysgenesis mesostromatolysis)
B. Essential iris atrophy (see p. 373)
C. Iris bombe from occlusion of pupil
D. Iris or ciliary body tumor pushing iris into contact with cornea
E. Local adhesion with of epithelium or fibrous ingrowth
F. Penetrating injury of the cornea
G. Postoperative flat anterior chamber
2. Synechiae of iris limited to ciliary band, scleral spur, and trabecular meshwork
(common)
*A. Following cataract surgery, intraocular implantation, refractive surgery, or
laser trabeculoplasty
*B. Intraocular inflammation
*C. Neovascular glaucoma from fibrovascular membrane (see p. 324)
*D. Sequelae to angle-closure glaucoma
Kolker AE, Hetherington J. Becker-Shaffer's diagnosis and therapy of the glaucomas, 6th
ed. St. Louis: CV Mosby, 1989.
1991.
Rouhiainen HJ, et al. Peripheral anterior synechiae formation after trabeculoplasty. Arch
Ophthalmol 1988; 106: 189-191.
NEOVASCULARIZATION OF ANTERIOR CHAMBER ANGLE (NEWLY

FORMED VESSELS EXTEND INTO THE TRABECULAR MESHWORK)
1. Anterior chamber angle

A. Congenital pupillary iris lens membrane with goniodysgenesis
B. Traumatic chamber angle
2. Iris tumors
A. Hemangioma
B. Melanoma
C. Metastatic carcinoma
3. Ocular vascular disease
*A. Central retinal artery thrombosis
*B. Central retinal vein thrombosis (see p. 468)
C. Hemiretinal branch vein occlusion (HBVO)
4. Postinflammatory
A. Anterior chamber implants
B. Fungal endophthalmitis
C. Radiation
D. Retinal detachment operation
E. Uveitis, chronic
5. Proximal vascular disease
A. Aortic arch syndrome (Takayasu syndrome)
B. Carotid cavernous fistula
C. Carotid ligation
D. Carotid occlusive disease
E. Cranial arteritis (temporal arteritis syndrome)
6. Retinal disease
A. Coats disease (Leber miliary aneurysms)
*B. Diabetic retinopathy
C. Eales disease (periphlebitis)
D. Glaucoma, chronic
E. Melanoma of choroid
F. Norrie disease (fetal iritis syndrome)
G. Persistent hyperplastic primary vitreous
H. Retinal detachment
I. Retinal hemangioma
J. Retinal vessel occlusion
K. Retinoblastoma
L. Retrolental fibroplasia
M. Sickle cell retinopathy (Herrick syndrome)
Cibis GW, et al. Congenital pupillary iris-lens membrane with goniodysgenesis.

Kimura R. Fluorescein gonioangiography of newly formed vessels in the anterior

chamber angle. Tohoku J Exp Med 1983; 140:193-196.
Shihab ZM, Lee PF. The significance of normal angle vessels. Ophthalmic Surg Lasers
1985; 16:382-385.
IRIS PROCESSES (PECTINATE LIGAMENTS IN ANTERIOR

CHAMBER ANGLE)
1. Achondroplasia, diastrophic dwarfism, cartilage-hair hypoplasia, and
spondyloepiphyseal dysplasia, anterior chamber cleavage syndrome, Axenfeld syndrome,
Reiger syndrome, Peter anomaly.
2. Congenital glaucoma-may be associated with congenital microcoria and
goniodysgenesis
3. Congenital scoliosis
4. Legg-Perthes disease (coxa plana)
5. Marfan syndrome (hypoplastic form of dystrophia mesodermalis congenita)
6. Mucopolysaccharidoses (including Hunter syndrome, Hurler syndrome, Scheie
syndrome, and Sanfilippo-Good syndrome)
7. Myopic patients
*8. Normal, especially in brown-eyed persons
9. Pigmentary ocular dispersion syndrome
Dunn SP, et al. New findings in posterior amorphous corneal dystrophy. Arch
Ophthalmol 1984; 102:236-239.
Pollock A, Oliver M. Congenital glaucoma and incomplete congenital glaucoma in two

siblings. Acta Ophthalmol Scand 1984; 62:359-363.
WIDTE MASS IN ANTERIOR CHAMBER
* 1. Endophthalmitis
2. Ocular aspergillosis
3. Sterile inflammation following surgery or trauma
*4. Tumor
Katz G, et al. Ocular aspergillosis isolated in the anterior chamber. Ophthalmology 1993;
100:1815-1818.
11
Pupil
CONTENTS
Mydriasis 344
Relative fixed, dilated pupil 348
Miosis 349
Paradoxical pupillary reaction 352
Absence or decrease of pupillary reaction to light 353
Anisocoria 354
Irregularity of pupil 355
Hippus 356
Tonohaptic pupil 356
Leukokoria 357
Long ciliary processes extending into dilated pupillary space 361
Persistent pupillary membrane 361
Decentered pupillary light reflex 361
Pupillary block following cataract extraction 361
Afferent pupillary defect 362
MYDRIASIS (DILATED PUPIL, USUALLY> 5 MM)
1. Physiologic
A. Larger pupils in women than in men
B. Larger pupils in myopes than in hypermetropes
C. Larger pupils in blue irides than in brown irides
D. Larger pupils in adolescents and middle-aged persons than in very young or
old persons
E. Surprise, fear, pain, strong emotion, or vestibular stimulation
F. General anesthesia of stages I, II, and IV
G. Autosensory pupillary reflex-stimulation of middle ear
H. Auditory pupillary reflex-tuning fork adjacent to ear
I. Vestibular pupillary reflex-stimulation of labyrinth by heat, cold, or rotation
J. Vagotonic pupillary reflex-stimulation on deep inspiration
acetaminophen allobarbital anisotropine
acetanilid alprazolam antazoline
acetophenazine alseroxylon antimony lithium
acetylcholine amantadine thiomalate
adiphenine ambutonium antimony potassium
adrenal cortex injection amitriptyline tartrate
albuterol amobarbital antimony sodium
alcohol amoxapine antimony sodium
aldosterone amphetamine tartrate
alkavervir amyl nitrite aprobarbital
aspirin clonidine epinephrine
atropine clorazepate ergot
atropine methylnitrate cocaine erythromycin
azatadine codeine ether
baclofen colistimethate ethopropazine
barbital colistin fenfluramine
belladonna contraceptives fludrocortisone
benzathine cortisone fluorometholone
benzathine cryptenamine fluphenazine
benzphetamine cyclizine fluprednisolone
benztropine cyclobarbital flurazepam
betamethasone cyclopentobarbital fluvoxamine
biperiden cyclopentolate hydrochloride
botulinum A cycrimine gentamicin (?)
bromide cyproheptadine glutethimide
bromisovalum deserpidine glycopyrrolate
brompheniramine desipramine guanethidine
butabarbital desoxycorticosterone halazepam
butalbital dexamethasone haloperidol
butallylonal dexbrompheniramine hashish
butaperazine dexchlorpheniramine heptabarbital
butethal dextroamphetamine hexachlorophene
caramiphen diacetylmorphine hexamethonium
carbamazepine diazepam hexethal
carbinoxamine dicyclomine hexobarbital
carbon dioxide diethazine hexocyclium
carbromal diethylpropion homatropine
carisoprodol digitalis hydrabamine
carphenazine digoxin penicillin V
chloral hydrate dimethindene hydrabamine
chloramphenicol diphemanil phenoxymethyl
chlorcyclizine diphenhydramine penicillin
chlordiazepoxide diphenylhydantoin hydrocortisone
chlorisondamine diphenylpyraline hydromorphone
chloroform diphtheria toxoid hydroxyamphetamine
chlorpheniramine (adsorbed) imipramine
chlorphenoxamine dipivalyl epinephrine indomethacin (?)
chlorphentermine (DPE) insulin
chlorpromazine dipivefrin iodide and iodine
chlorprothixene disopyramide solutions and
cimetidine disulfiram compounds
clemastine doxepin isocarboxazid
clidinium doxylamine isoniazid
clomiphene droperidol isopropamide
clomipramine emetine levallorphan
clonazepam ephedrine levarterenol
levodopa metoclopramide pilocarpine
lidocaine midazolam pipenzolate
lorazepam morphine piperacetazine
loxapine mumps virus vaccine piperidolate
lysergic acid (live) poldine
diethylamide (LSD) nalidixic acid polymyxin B
lysergide nalorphine potassium penicillin G
maprotiline naloxone potassium penicillin V
marijuana naltrexone potassium phenethicillin
measles and rubella naphazoline potassium
virus vaccine (live) nialamide phenoxymethyl
measles virus vaccine nitrazepam penicillin
(live) nitroglycerin (?) prazepam
measles, mumps, and nitrous oxide prednisolone
rubella virus vaccine norepinephrine prednisone
mecamylamine nortriptyline primidone
meclizine opium probarbital
medrysone oral contraceptives procaine penicillin G
mepenzolate orphenadrine prochlorperazine
meperidine oxazepam procyclidine
mephentermine oxygen promazine
mephobarbital oxymorphone promethazine
meprednisone oxyphencyclimine propantheline
meprobamate oxyphenonium propiomazine
mescaline paraldehyde propoxyphene
mesoridazine paramethasone protoveratrines A and B
metaraminol pargyline protriptyline
methadone penicillin G psilocybin
methamphetamine pentobarbital pyrilamine
methantheline pentolinium quinidine
methaqualone pentylenetetrazol quinine
metharbital perazine radioactive iodides
methdilazine pericyazine rauwolfia serpentina
methitural perphenazine rescinnamine
methixene phenacetin reserpine
methohexital phendimetrazine rubella and mumps virus
methotrimeprazine phenelzine vaccine (live)
methoxamine pheniramine rubella virus vaccine
methscopolamine phenmetrazine (live)
methyl alcohol phenobarbital scopolamine
methylatropine nitrate phenoxymethyl secobarbital
methylene blue penicillin sodium
methylpentynol phentermine antimonylgluconate
methylphenidate phenylephrine sodium salicylate
methylprednisolone phenylpropanolamine stibocaptate
methyprylon phenytoin stibogluconate
stibophen thiopropazate trihexyphenidyl
syrosingopine thioproperazine trimeprazine
talbutal thioridazine trimethaphan
temazepam thiothixene trimethidinium
tetraethylammonium tolazoline trimipramine
tetrahydrocannabinol tranylcypromine tripelennamine
tetrahydrozoline trazodone tropicamide
thiamylal triamcinolone urethan
thiethylperazine triazolam veratrum viride
thiocarbanidin tridihexethyl alkaloids
(THC) (?) trifluoperazine vinbarbital
thioglycollate trifluperidol
thiopental triflupromazine
3. Toxins, including after-shave lotion, arsenic, Clostridium botulinum (gas gangrene),

tetanus (lockjaw), cannabis, adrenergic agents (such as nasal sprays or asthma therapy in
newborns), paraaminosalicylic acid, lead, carbon monoxide, organic phosphorus, bovine
milk protein in infants with allergic malabsorption, Datura stramonium (Jimson weed),
Datura wrightii (moonflower), and Solanaceae (nightshade), nitrocompounds and
aminocompounds of benzene, carbon disulfide, and papaverine.
4. Ocular causes (mydriasis) (see fixed pupil section p. 348)
A. Glaucoma, usually acute
B. Glaucomocylitic crisis (Posner-Schlossman syndrome)
C. Hollenhorst syndrome (chorioretinal infarction syndrome)
*D. Iritis; uveitis
E. Intraocular foreign body (iron mydriasis)
F. Iris atrophy
G. Iris sphincter rupture
*H. Paralytic mydriasis following trauma
I. Photocoagulation complications
J. Retinoblastoma
5. Lesions of ciliary ganglion causing internal ophthalmoplegia (e.g., dilated pupil and
absent accommodation)
A. Adie tonic pupil
B. Congenital lesion
C. Herpes zoster
D. Orbital floor fracture repair
E. Systemic lupus erythematosus (disseminated lupus erythematosus)
F. Varicella (chickenpox)
G. Yellow fever
6. Acute or chronic ophthalmoplegias (see p. 168-171)
7. Third-nerve lesion-also ptosis and ophthalmoplegia on affected side (see p. 168-171)
8. Coma because of alcohol ingestion, eclampsia, diabetes, uremia, epilepsy, apoplexy, or
meningitis-the pupils are equally dilated and do not constrict with stimulation
9. Midbrain tumors, in which dilated pupils, paralysis of vertical gaze (especially upward
gaze), and retraction nystagmus are manifested
A. Craniopharyngioma
B. Parinaud syndrome (paralysis of upgaze movements)
10. Epidural or subdural hematoma
11. Paralytic parasympathetic lesions
12. Irritative sympathetic lesion-pupillary dilatation widening of palpebral aperture and
slight exophthalmos
A. Irritative lesion, such as tumor, encephalitis, or syringomyelia of the
hypothalamus, midbrain, medulla, or cervical cord
B. Thoracic lesions, such as cervical rib, aneurysms of the thoracic vessels,
mediastinal tumors, or tubercular pleurisy
*C. Cervical lesions, including nasopharyngeal tumors, thyroid swelling, or
cervical nodes
D. Rabies (hydrophobia)
*E. Trauma
F. Visceral disease
G. Aortic dilatation or exudative endocarditis (Roque sign)
H. Acute abdominal conditions, such as appendicitis, cholecystitis, or colitis
(Moschowitz sign)
I. Psychiatric patients with pressure over McBurney point (Meyer phenomenon)
13. Tumors, injury, or hemorrhage of frontoparietal, parietal, temporal, or
temporooccipital area-contralateral mydriasis and ipsilateral defect in the visual field
14. Fractured skull
15. Acute autonomic neuropathy
16. Acute pandysautonomia
17. Avitaminosis Bz (pellagra)
18. Chorea
19. Clivus edge syndrome
21. Foramen lacerum syndrome (aneurysm of internal carotid artery syndrome)
22. Hemiacrosomia syndrome (hemifacial or unilateral hypertrophy)
23. Iron deficiency anemia
24. Lockjaw (tetanus)
25. Mycosis fungoides syndrome (Sézary syndrome)
26. Optic canal syndrome
27. Parkinson syndrome (shaking palsy)
28. Prematurity
29. Pulseless disease
30. Reye syndrome (acute encephalopathy syndrome)
32. Suprarenal-sympathetic syndrome (adrenal medulla tumor syndrome)
34. Weber syndrome (cerebellar peduncle syndrome)
35. Wernicke syndrome (I) (avitaminosis Bl thiamine deficiency)
36. Zellweger syndrome (cerebrohepatorenal syndrome)
Bodker FS, et al. Postoperative mydriasis after repair of orbital floor fracture. Am J
Ophthalmol, 1993; 115:372-375.
Cuppeto JR, Greco T. Mydriasis in giant cell arteritis. J Clin Neuroophthalmol 1985;
9:267.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects. Woburn, MA

Hendrix LE, et al. Papaverine-induced mydriasis. AJNR Am J Neuroradiol 1994; 15:716-

718.
Isenberg SJ, et al. The fixed and dilated pupils of premature neonates. Am J Ophthalmol
1990; 110: 168.
Richardson P, Schulenburg WE. Bilateral congenital mydriasis. Br J Ophthalmol 1992;

76:632-633.
RELATIVE FIXED, DILATED PUPIL
1. Midbrain damage-vascular accidents, tumors, degenerative and infectious diseases

A. Dorsal (Edinger-Westphal nucleus and its connections)-rare, involves both
pupils, pupillary near reaction often retained, and often associated with
supranuclear vertical gaze palsy (upgaze)
*B. Ventral (fascicular part of third nerve)-associated with other neurologic
deficits, such as Nothnagel syndrome, Benedikt syndrome, Weber syndrome, and
involves other extraocular components of the third nerve
*2. Damage to the third nerve (from interpeduncular fossa to ciliary ganglion)
A. Basal aneurysms
B. Supratentorial space-occupying masses, causing displacement of the brainstem
or transtentorial herniation of the uncus; patient is stuporous or comatose
C. Basal meningitis-often bilateral internal ophthalmoplegia
*D. Ischemic oculomotor palsy
E. Parasellar tumor (e.g., pituitary adenoma, meningioma, craniopharyngioma,
nasopharyngeal carcinoma, or distant metastases)
F. Parasellar inflammation (e.g., Tolosa-Hunt syndrome, temporal arteritis, herpes
zoster)
*3. Damage to the ciliary ganglion
A. Viral ciliary ganglionitis or involvement of the ciliary nerves, such as from
herpes zoster
B. Orbital trauma or tumor
C. Trauma from inferior oblique surgery
D. Trauma from retrobulbar injections
4. Damage to short ciliary nerves
A. Blunt trauma to the globe may injure the ciliary plexus at the iris root
(traumatic iridoplegia)
B. Choroidal trauma or tumor
*5. Damage to the iris
A. Degenerative or inflammatory diseases of the iris
B. Posterior synechiae
C. Acute rise of intraocular pressure (hypoxia or sphincter damage)
D. Blunt injury to the globe with sphincter damage (traumatic iridoplegia)
E. Pharmacologic blockade by atropinic substances
F. Following cataract surgery
6. Total blindness, including cortical blindness (see p. 632-636)
A. Bilateral optic nerve
(1) Anterior ischemic optic neuropathy
(2) Avulsion (traumatic)
(3) Optic neuritis
B. Bilateral retina
(1) Acute retinal necrosis
(2) Central retinal artery occlusion
(3) Central retinal vein occlusion
(4) Retina detachment
Isenberg SJ, et al. The fixed and dilated pupils of premature neonates. Am J Ophthalmol
1990; 110: 168-171.
Lam S, et al. Atonic pupil after cataract surgery. Ophthalmology 1989; 96:589-590.
Newell FW. Ophthalmology: principles and concepts. 7th ed. St. Louis: CV Mosby,
1991.
Thompson HS, et al. The fixed dilated pupil. Arch Ophthalmol 1971; 86:21-27.
MIOSIS (SMALL PUPIL) (USUALLY <2 MM)
1. Physiologic
A. Smaller pupil in men than in women
B. Smaller pupil in hypermetropes than in myopes
C. Smaller pupil in brown irides than in blue irides
D. Smaller pupil in very young or old than in adolescents and middle-aged
persons
E. Sleep, fatigue, coma
F. Stage III anesthesia
G. Near vision (synkinesis with convergence and accommodation)
H. Vestibular stimulation
aceclidine edrophonium neostigmine
acetophenazine ephedrine (?) nialamide
acetylcholine ergot nitrous oxide
alcohol ergotamine opium
allobarbital ether oxprenolol
alseroxylon ethopropazine oxymorphone
ambenonium fluphenazine paraldehyde
amobarbital guanethidine pentazocine
aprobarbital haloperidol pentobarbital
baclofen hashish perazine
barbital heptabarbital pericyazine
bethanechol hexachlorophene perphenazine
bromide hexethal phencyclidine
bromisovalum hexobarbital phenelzine
bupivacaine hydromorphone phenobarbital
butabarbital indomethacin phenoxybenzamine
butalbital iodide and iodine phenylephrine
butallylonal solutions and physostigmine
butaperazine compounds (?) pilocarpine
butethal isocarboxazid piperacetazine
carbachol isoflurophate piperazine
carbromal isosorbide dinitrate (?) piperocaine
carisoprodol levallorphan prilocaine
carphenazine levodopa primidone
chloral hydrate lidocaine probarbital
chloroform marijuana procaine
chloroprocaine meperidine prochlorperazine
chlorpromazine mephobarbital promazine
chlorprothixene mepivacaine promethazine
clonidine meprobamate propiomazine
codeine mesoridazine propoxycaine
cyclobarbital methacholine propoxyphene
cyclopentobarbital methadone propranolol
demecarium methaqualone (?) pyridostigmine
deserpidine metharbital radioactive iodides (?)
diacetylmorphine methdilazine rauwolfia serpentina
dibucaine methitural rescinnamine reserpine
diethazine methohexital secobarbital
digitalis(?) methotrimeprazine sulindac
diisopropyl methyprylon syrosingopine
flurophosphate midazolam talbutal
(DFP) morphine tetracaine
dronabinol nalorphine tetrahydrocannabinol
droperidol naloxone thiamylal
echothiophate naltrexone thiethylperazine
thiopental tolazoline trimeprazine
thiopropazate tranylcypromine vinbarbital
thioproperazine trifluoperazine vitamin A
thioridazine trifluperidol
thiothixene triflupromazine
3. Ocular causes
*A. Accommodative spasm (hysteria)
B. Corneal irritation, such as keratitis or corneal injury
C. Conjunctival irritation
D. Congenital miosis (absent dilator muscle)
E. Dislocated lenses
F. Iritis
*G. Posterior iris synechiae, usually irregular
H. Retinitis pigmentosa
4. Central nervous system defects
A. Acute pontine angle lesion, such as hemorrhage or tumor associated with
disturbed conjugate gaze
B. Arteriosclerotic and degenerative disease of the cerebrum
C. Encephalitis
D. Facial tetanus
E. Giant cell (temporal arteritis)
F. Infections or tumors of the cavernous sinus or superior orbital fissure
G. Purulent meningitis
H. Severe hypoxia
5. "Cluster headache" or histamine cephalgia-ptosis; miosis; red, watering eye on side of
headache
6. Raeder paratrigeminal syndrome-ipsilateral miosis and pain-may be associated with
third-nerve paralysis or corneal anesthesia
A. Extracranial aneurysm of internal carotid
B. Idiopathic
C. Meningioma
D. Migraine
E. Posttrauma
7. Argyll Robertson pupil-small and irregular; reacts better to accommodation than to
light
A. Aberrant regeneration of the third nerve
B. Carbon disulfide poisoning
C. Cerebral aneurysm
D. Chronic alcoholism
*E. Diabetes mellitus (Willis disease)
F. Encephalitis
G. Friedreich ataxia
H. Malaria
I. Midbrain tumors, such as pinealomas and craniopharyngioma
J. Multiple sclerosis (disseminated sclerosis)
K. Senile and degenerative diseases of the central nervous system
L. Syphilis (acquired lues)
M. Syringomyelia
N. Trauma to skull or orbit
8. Ataxia, spastic with congenital miosis-dominant
9. Babinski-Nageotte syndrome (medulla tegmental paralysis)
10. Coenurosis
12. Dejerine-Klumpke syndrome (lower radicular syndrome)
13. Devic syndrome (neuromyelitis optica)
15. Eaton-Lambert syndrome (myasthenic syndrome)
16. Elevated intracranial pressure
*17. Homer syndrome (cervical sympathetic paralysis syndrome)
18. Jugular foramen syndrome (Vernet syndrome)
21. Morquio syndrome (mucopolysaccharidosis IV)
22. Myotonic dystrophy (Curschmann-Stewart syndrome)
23. Naffziger syndrome (scalenus anticus syndrome)
24. Pancoast syndrome (superior pulmonary sulcus syndrome)
25. Parkinsonism (shaking palsy)
26. Psychogenic diseases, such as schizophrenia, dementia precox, or hysteria
28. Retroparotid space syndrome (Villaret syndrome)
29. Romberg syndrome (facial hemiatrophy)
30. Spider bites
31. Stormorken syndrome (thrombocytopathia bleeding tendency)
32. Tetanus (lockjaw)
33. von Herrenschwand syndrome (sympathetic heterochromia)
34. Wallenberg syndrome (dorsolateral medullary syndrome)
35. Wernicke syndrome (avitaminosis B1, thiamine deficiency)
Ghanchi F, Dutton GN. Current concepts in giant cell (temporal) arteritis. Surv
Ophthalmol 1997; 42:99-l23.
PARADOXICAL PUPILLARY REACTION (CONSTRICTS WHEN LIGHT IS

WITHDRAWN)
1. Best disease
*2. Congenital achromatopsia
*3. Congenital stationary night blindness
5. Optic nerve hypoplasia
Barricks ME, et al. Paradoxical pupillary responses in congenital stationary night

blindness. Arch Ophthalmol 1977; 95:1800-1804.
Flynn JT, et al. Paradoxical pupil in congenital achromatopsia. Int Ophthalmol 1981;
2:91-96.
Frank JW, et al. Paradoxic pupillary phenomena. Arch Ophthalmol 1988; 106:l564.
ABSENCE OR DECREASE OF PUPILLARY REACTION TO LIGHT
This type of absence or decreased pupillary reaction to light is caused by drugs,
including the following:
acetaminophen bromisovalum clorazepate
acetanilid brompheniramine cloxacillin (?)
acetophenazine butabarbital cocaine
alcohol butalbital cyclizine
allobarbital butallylonal cyclobarbital
alprazolam butaperazine cyclopentobarbital
amitriptyline butethal cycrimine
amobarbital calcifediol desipramine
amoxapine calcitriol dexbrompheniramine
amoxicillin carbenicillin (?) dexchlorpheniramine
amphetamine carbinoxamine dextroamphetamine
ampicillin carbon dioxide diacetylmorphine
antazoline carbromal diazepam
antimony lithium carisoprodol dicloxacillin (?)
thiomalate carmustine diethazine
antimony potassium carphenazine dimethindene
tartrate chloramphenicol diphenhydramine
antimony sodium chlorcyclizine diphenylpyraline
tartrate chlordiazepoxide diphtheria toxoid,
antimony sodium chloroprocaine adsorbed
thioglycollate chlorpheniramine doxepin
aprobarbital chlorphenoxamine doxylamine
aspirin chlorpromazine emetine
atropine chlorprothixene ergocalciferol
baclofen cholecalciferol ergot
barbital cimetidine ethopropazine
belladonna clemastine etidocaine
benztropine clomipramine fenfluramine
biperiden clonazepam fluphenazine
bromide clonidine flurazepam
glutethimide nialamide ranifidine
halazepam nitrazepam scopolamine
heptabarbital nortriptyline secobarbital
hetacillin (?) orphenadrine sodium
hexachlorophene oxacillin (?) antimonylgluconate
hexethal oxazepam sodium salicylate
hexobarbital pargyline stibocaptate
homatropine pentobarbital stibogluconate
imipramine pentylenetetrazol stibophen
insulin perazine talbutal
isocarboxazid periciazine temazepam
isoniazid perphenazine tetanus immune globulin
lidocaine phenacetin tetanus toxoid
lorazepam phencyclidine thiamylal
LSD phenelzine thiethylperazine
lysergide pheniramine thiopental
meclizine phenmetrazine thiopropazate
meperidine phenobarbital thioproperazine
mephobarbital phenylpropanolamine thioridazine
meplvacaine phenytoin thiothixene
meprobamate piperacetazine tranylcypromine
mescaline prazepam triazolam
mesoridazine prilocaine trichloroethylene
methamphetamine primidone trifluoperazine
methaqualone probarbital triflupromazine
metharbital procaine trihexyphenidyl
methdilazine prochlorperazine trimeprazine
methicillin (?) procyclidine trimipramine
methitural promazine tripelennamine
methohexital promethazine triprolidine
methotrimeprazine propantheline urethan
methscopolamine propiomazine vinbarbital
methyl alcohol propoxycaine vitamin D
methyprylon protriptyline vitamin D2
midazolam psilocybin vitamin D3
nafcillin (?) pyrilamine
neomycin quinine
ANISOCORIA (INEQUALITY OF PUPILS OF ≥1 MM)
1. Central nervous system

*A. Adie (tonic) pupil
B. Aneurysm of the aorta or carotid artery
C. Cerebrovascular accidents
D. Cervical rib (ipsilateral constricted pupil)
E. Encephalitis (mild cases)
*F. Homer syndrome (cervical sympathetic paralysis syndrome)
G. Pontine lesions
H. Tabes dorsalis
I. Third-nerve paresis
J. Trigeminal neuralgia (tic douloureux)
K. Wernicke hemianopic pupil
alcohol diphenylpyraline oral contraceptives
antazoline disulfiram phenelzine
bromide doxylamine pheniramine
bromisovalum dronabinol phenylpropanolamine
brompheniramine ethchlorvynol prilocaine
bupivacaine etidocaine procaine
carbinoxamine hashish propoxycaine
carbromal isocarboxazid psilocybin
chloroprocaine jimsonweed pyrilamine
chlorpheniramine lidocaine scopolamine
clemastine LSD tetrahydrocannabinol
contraceptives lysergide tranylcypromine
dexbrompheniramine marijuana trichloroethylene
dexchlorpheniramine mepivacaine tripelennamine
diacetylmorphine mescaline triprolidine
dimethindene methaqualone
diphenhydramine nialamide
3. Ocular conditions
A. Artificial eye (pseudoanisocoria)
B. Cornea, such as keratitis or abrasion
C. Glaucoma, including pigmentary dispersion
*D. Iris, such as iritis, synechiae, iris atrophy, or iris sphincter rupture
E. Ocular trauma
F. Spastic miosis
4. Physiologic
A. Anisometropia-larger pupil with the more myopic eye
B. Familial
C. Lateral illumination of one eye gives more miosis in that eye than in the other
D. Nonfamilial-normal variation (small percentage of the population)
E. Tournay reaction-with the eyes turned sharply to the side, dilatation of the
pupil of the abducting eye and miosis of pupil of the adducting eye
5. Unilateral miosis (see p. 349)
6. Unilateral mydriasis (see p. 349)
Cheng MM, Catalano RA. Fatigue-induced familial anisocoria. Am J Ophthalmol 1990;
109:480-481.
Feibel RM, Perlmutter JC. Anisocoria in the pigmentary dispersion syndrome. Am J

Nakagawa TA, et al. Aerosolized atropine as an unusual cause of anisocoria in a child

with asthma. Pediatr Emerg Care 1993; 9:153-154.
IRREGULARITY OF PUPIL (INCLUDING OVAL OR PEAKED PUPIL)
1. Adherent leukoma as one part of iris is pulled up to corneal scar, peripheral anterior
synechiae, or corneal laceration with prolapse of iris
3. Anterior chamber intraocular lens that is too long or erodes into uveal tissue
4. Argyll-Robertson pupil-small and irregular; reacts better to accommodation than to
light; same type as seen in diabetic patients (pseudodiabetic pupil)
5. Congenital coloboma of the iris, usually below
6. Following laser iridectomy
7. Glaucoma-oval, dilated pupil
*8. Injury of the iris
9. Iris tuck of anterior chamber intraocular lens
*10. Iritis-usually small but pupil may be any shape with anterior or posterior synechiae
*11. Long -term intraocular inflammation
12. Medication, with faster reaction of one sector of iris than of another-miosis or
mydriasis
13. Operation-as sector iridectomy or peripheral iridectomy
14. Optic atrophy due to causes such as syphilis, quinine poisoning, and internal
ophthalmoplegia of vascular or traumatic origin
15. Piece of anterior capsule into anterior chamber
16. Posterior chamber intraocular lens with loop of intraocular lens holding the
midportion of iris peripherally
17. Posterior chamber lens with two haptics having the lens either behind the pupil with
the haptics in front or having the lens anterior to the pupil with the haptics behind the iris
18. Segmental iris atrophy
19. Tumors of iris or ciliary body
20. Vitreous or zonules into corneal laceration
21. Vitreous strand from behind pupil to wound
22. Wound leak with or without prolapse of the iris
Fuller JR. Iris creep producing correctopia in response to Molteno implants. Arch
Ophthalmol 2001; 119:304-306.
Moster MR, et al. Laser iridectomy. Ophthalmology 1986; 93:20-24.
1991.
Reidy JJ, et al. An analysis of semiflexible, closed-loop anterior chamber intraocular

lenses. Am Intraocular Implant Soc J 1985; 11:344-352.
HIPPUS
Hippus is visible, rhythmic, but irregular pupillary oscillations that are deliberate
in time. It comprises 2 mm or more excursions and has no localizing significance.
1. Normal
2. Incipient cataracts
3. Central nervous system diseases, including the presence of total third cranial nerve
palsy, hemiplegia, meningitis (acute), cerebral syphilis, tabes, general paralysis,
myasthenia gravis, tumors of corpora quadrigemina, epileptics, Cheyne-Strokes
breathing, multiple sclerosis (disseminated sclerosis), and cerebral tumors
4. Neurasthenia (nervous exhaustion, Beard disease)
allobarbital cyclopentobarbital pentylenetetrazol
amobarbital heptabarbital phenobarbital
aprobarbital hexethal primidone
barbital hexobarbital probarbital
butabarbital mephobarbital secobarbital
butalbital metharbital talbutal
butallylonal methitural thiamylal
butethal methohexital thiopental
cyclobarbital pentobarbital vinbarbital
Zinn KM. The pupil. Springfield, IL: Charles C Thomas, 1972.
TONOHAPTIC PUPIL
Tonohaptic pupil involves a long latent period preceding both contraction to light
and redilatation, followed in each instance by a short but prompt movement.
1. Catatonic state
3. Diabetes insipidus
4. Dystrophia adiposogenitalis (Fröhlich syndrome) or pituitary cachexia (Simmonds
disease)
5. Introverted persons of the schizophrenic group
6. Parkinsonism (shaking palsy)
7. Pigmentary retinal dystrophy
8. Postencephalitic condition
9. Schizoid state
Duke-Elder S, Scott GI. System of ophthalmology, Vol 12. St. Louis: CV Mosby, 1971.
LEUKOKORIA (WHITE PUPIL) (SEE LESIONS CONFUSED WITH

RETINOBLASTOMA, P. 502)
1. Angiomatosis of retina (cerebelloretinal hemangioblastomatosis)

2. Astrocytoma
*3. Cataract (congenital)
4. Choroidal hemangioma
*5. Coats disease (retinal telangiectasia)
6. Coloboma of choroid and optic disc
7. Congenital cytomegalovirus retinitis
8. Congenital retinal detachment
9. Exudative retinitis, chorioretinitis, or both
10. Falciform fold of retina
11. Familial exudative vitreoretinopathy
12. Herpes simplex retinitis
13. High myopia with advanced chorioretinal degeneration
*14. Medullation of nerve fiber layer
15. Metastatic endophthalmitis
16. Morning glory syndrome (hereditary central glial anomaly of the optic disc)
17. Nematode endophthalmitis (Toxocara canis)
18. Norrie disease (atrophia oculi congenita)
*19. Ocular toxocariasis
20. Organized vitreous hemorrhage
22. Physiologic-eye photographed at 17 to 20 degrees temporal to fixation
23. Retinal dysplasia (massive retinal fibrosis)
*24. Retinoblastoma
25. Retinopathy of prematurity (ROP)
26. Retrolental membrane associated with Bloch-Sulzberger syndrome (incontinentia
pigmenti)
*27. Toxoplasmosis (congenital)
28. Traumatic chorioretinitis
29. Tumors other than retinoblastoma
A. Choroidal hemangioma
B. Combined retinal hamartoma
C. Diktyoma
D. Glioneuroma
E. Leukemia
F. Medulloepithelioma
G. Retinal astrocytic hamartoma
H. Retinal capillary hemangioma
30. Uveitis (peripheral)
31. Vitreous organization following unsuspected penetrating wounds
Chang-Godinich A, et al. Familial exudative vitreoretinopathy mimicking persistent

hyperplastic primary vitreous. Am J Ophthalmol 1999; 127, 4:469-471.
Federman JL, et al. The surgical and nonsurgical management of persistent hyperplastic
primary vitreous. Ophthalmology 1982; 89:20.
Ing E. Personal communication. December, 2001.
Shapiro DR, Stone RD. Ultrasonic characteristics of retinopathy of prematurity

presenting with leukokoria. Arch Ophthalmol 1985; 103:1690-l694.
Shields JA, et al. Malignant teratoid medulloepithelioma of the ciliary body simulating
persistent hyperplastic primary vitreous. Am J Ophthalmol 1989; 107:296-300.
Extracted Table Leukokoria (white pupil)
LONG CILIARY PROCESSES EXTENDING INTO DILATED PUPILLARY

SPACE
1. Aniridia
2. Anterior rotation of ciliary processes
A. After scleral buckling operation
B. Angle closure
C. Anterior choroidal separation
D. Cyst or tumor behind iris
E. Dislocated lens
F. From adherence to limbal scar
G. Plateau iris
3. Extreme mydriasis
4. Falciform detachment of the retina
5. Incontinentia pigmenti (Bloch-Sulzberger syndrome)
6. Norrie disease (atrophia oculi congenita)
7. Persistent hyperplastic primary vitreous (PHPV)
8. Retinal dysplasia of Reese
9. Retrolental fibroplasia (RLF)
10. Surgical coloboma
11. Trisomy 13 (trisomy D)
Hansen AC. Norrie's disease. Am J Ophthalmol 1963; 66:320-332.
PERSISTENT PUPILLARY MEMBRANE
1. Fetal iritis
2. Hereditary
3. Physiologic
*4. Use of oxygen therapy in nursery for premature infants
Hornblass A. Persistent pupillary membrane and oxygen therapy in premature infants.

Ann Ophthalmol 1971; 3: 95-99.
DECENTERED PUPILLARY LIGHT REFLEX
1. Positive angle kappa-pseudoexotropia

2. Negative angle kappa-pseudoesotropia
*3. Eccentric fixation-deep unilateral amblyopia
*4. Ectopic macula-macular displacement by retinal scarring or strands, such as
retrolental fibroplasia
5. Ectopic pupil
Beyer-Machule C, yon Noorden GK. Atlas of ophthalmic surgery, Vol 1: Lids, orbits,
PUPILLARY BLOCK FOLLOWING CATARACT EXTRACTION
1. Air pupillary block

2. Dense, impermeable anterior hyaloid membrane
3. Free vitreous block
4. Intraocular lens effectively closing off pupil and iridectomies
5. Leaky wound
6. Nonperforating iridectomy
7. Posterior vitreous detachment associated with pooling or retrovitreal aqueous
8. Postoperative iridocyclitis
9. Subchoroidal hemorrhage
10. Swollen lens material behind the iris
Tomey KF, Traverso CE, Neodymium-YAG laser posterior capsulotomy for the
treatment of aphakic and pseudophakic pupillary block. Am J Ophthalmol 1987; 104:502-
507.
AFFERENT PUPILLARY DEFECT

The pupil of the eye has diminished vision from disease of the retina or optic
nerve and will fail to react directly to light but will constrict consensually when the
healthy eye is stimulated.
1. Amblyopia (rare)
2. Branch retinal artery/vein occlusion
3. Central retinal artery/vein occlusion
4. Compressive optic neuropathy
A. Cavernous hemangiomas
B. Cystic tumors
(1) Cholesterol granuloma
(2) Conjunctival orbital cysts
(3) Dermoid cysts
(4) Mucoceles
C. Inflammatory and infiltrative processes
D. Optic nerve tumors
(1) Optic nerve gliomas
(2) Optic nerve meningiomas
E. Primary malignancies
F. Sarcoidosis
G. Solid orbital tumors
(1) Hemangiomas
(2) Meningiomas
(3) Schwannoma
H. Thyroid ophthalmopathy
I. Trauma
5. Diabetic retinopathy (severe)
6. Hyphema
7. Macular degeneration (rarely)
8. Neovascular glaucoma
*9. Optic neuritis
*10. Optic nerve lesion
11. Radiation
12. Reticulum cell sarcoma
14. Toxoplasma retinochoroiditis
15. Traumatic optic neuropathy and retinopathy
16. Unilateral optic nerve hypoplasia
Browning DJ, Tiedeman JS. The test light affects quantitation of the afferent pupillary
defect. Ophthalmology 1987; 94:53-55.
Burde RM, et al. Clinical decisions in neuro-ophthalmology, 2nd ed. St. Louis: CV
Mosby, 1991.
Enyedi LB, et al. A comparison of the Marcus Gunn and alternating light tests for
afferent pupillary defects. Ophthalmology 1998; 105:871-873.
Girkin CA, et al. A relative afferent pupillary defect without any visual sensory deficit.
Arch Ophthalmol 1998; 1544-1547.
12
Iris
CONTENTS
Aniridia 364
Coloboma of iris 365
Rubeosis iridis 366
Hyperemia of iris 367
Heterochromia 368
Hyperchromic heterochromia 368
Iris atrophy 373
Iridodonesis 374
Tumors arising from pigment epithelium of the iris 374
Pigmented lesions of the iris 374
Nonpigmented lesions of the iris 375
Conditions simulating anterior uveitis or iritis 376
Syndromes and diseases associated with iritis 377
Iritis (anterior uveitis) in children 378
Nongranulomatous uveitis 381
Granulomatous uveitis 382
Pigmented ciliary body lesions 384
Neuroepithelial tumors of ciliary body 384
Internal ophthalmoplegia 385
ANIRIDIA (ABSENCE OF IRIS, PARTIAL OR COMPLETE)
1. AGR triad-sporadic (bilateral or unilateral) aniridia, genitourinary abnormalities, and

mental retardation
A. Cataracts
B. Corneal dystrophy
C. Ectopia lentis
D. Glaucoma
*E. Macular aplasia - autosomal dominant
F. Microcornea and subluxated lenses
G. Nystagmus
H. Optic nerve hypoplasia
I. Photophobia
J. Poor foveal reflex
*K. Strabismus
3. Associated with autosomal-recessive inheritance with fully developed macula
4. Associated with unilateral renal agenesis and psychomotor retardation
5. Beckwith-Wiedemann syndrome
6. Deletion of short arm of 11th chromosome
7. Gillespie syndrome (incomplete aniridia, cerebellar ataxia, and oligophrenia)
8. Homocystinuria syndrome
9. Marinesco-Sjögren syndrome (congenital spinocerebellar ataxia)
10. Miller syndrome (Wilms aniridia syndrome)
11. Partial trisomy 2q
12. Peters syndrome (oculodental syndrome)
14. Ring chromosome 6
16. Siemens syndrome (anhidrotic ectodermal dysplasia)
*17. Traumatic
18. Ullrich syndrome (dyscraniopylophalangy)
Nelson IB, et al. Aniridia: a review. Surv Ophthalmol 1984; 28:621-642.
Pearce WG. Variability of iris defects in autosomal dominant aniridia. Can J Ophthalmol
1994; 29:25-29.
COLOBOMA OF IRIS
This condition involves failure of fusions of fetal fissure in optic vesicle, usually
inferior or inferonasal.
1. Acrorenoocular syndrome
2. Aicardi syndrome
3. Aniridia
4. Biemond syndrome
5. Cat eye syndrome (partial G-trisomy syndrome)
6. CHARGE association (coloboma, heart anomaly, choanal atresia, retardation, genital,
and ear anomalies)
7. Chromosome partial short-arm deletion syndrome
10. Focal dermal hypoplasia syndrome (Goltz syndrome)
11. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial syndrome)
12. Hemifacial microsoma syndrome (otomandibular dysostosis)
*13. Hereditary usually dominant may be recessive
14. Hurler syndrome (mucopolysaccharidoses I)
15. Hyperchromic heterochromia
16. Jeune disease (asphyxiating thoracic dystrophy)
17. Joubert syndrome
18. Kartagener syndrome
19. Klinefelter syndrome
20. Klippel-Trenaunay-Weber syndrome (angioosteohypertrophy syndrome)
21. Langer-Giedion syndrome
22. Lanzieri syndrome
23. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosapolydactyly-
*24. Marfan syndrome (dolichostenomelia-arachnodactyly-hyperchondroplasia-
dystrophia mesodermalis congenita)
25. Maternal use of thalidomide
26. Maternal vitamin A deficiency
27. Meckel syndrome
28. Median facial cleft syndrome
29. Microphthalmos syndrome (Meyer-Schwickerath and Weyers syndrome)
30. Nevoid basal cell carcinoma syndrome
31. Nevus sebaceous of Jadassohn (linear sebaceous nevus syndrome of Jadassohn)
32. Obesity -cerebral-ocular -skeletal anomalies syndrome
33. Oculoauriculovertebral dysplasia syndrome
35. Otomandibular dysostosis (hemifacial microsomia syndrome)
36. Partial deletion of group D chromosome
37. Rieger syndrome (dysgenesis mesodermalis corneae et irides)
39. Rubinstein-Taybi syndrome (broad-thumbs syndrome)
*40. Sporadic
41. Treacher Collins syndrome (Franceschetti syndrome)
42. Trisomy 13 (D trisomy) (Patau syndrome)
43. Trisomy 17 syndrome (Edwards syndrome)
44. Turner syndrome
45. Warburg syndrome
46. White sponge nevus
48. 11q syndrome
49. 13q syndrome
50. 13r syndrome
51. 18q syndrome
52. 18r syndrome
53. XYY syndrome
RUBEOSIS IRIDIS (NEOVASCULARIZATION [NEWLY FORMED BLOOD

VESSELS] ON THE IRIS)
1. Proximal vascular disease

A. Aortic arch syndrome (pulseless disease; Takayasu syndrome)
B. Carotid-cavernous fistula (carotid artery syndrome)
C. Carotid ligation
D. Carotid occlusive disease
E. Cranial arteritis syndrome (giant cell arteritis)
2. Ocular vascular disease
*A. Central retinal artery thrombosis (see p. 457)
*B. Central retinal vein thrombosis (see p. 467)
C. Long posterior ciliary artery occlusion
D. Reversed flow through the ophthalmic artery
3. Retinal diseases
A. Coats disease (retinal telangiectasia)
*B. Diabetes mellitus
C. Eales disease (periphlebitis)
D. Glaucoma, chronic
E. Melanoma of choroid
F. Norrie disease (oligophrenia-microphthalmos syndrome)
G. Persistent hyperplastic primary vitreous
H. Retinal detachment
I. Retinal hemangioma
J. Retinoblastoma
K. Retrolental fibroplasia
L. Sickle cell disease (Herrick syndrome)
4. Iris tumors
A. Hemangioma
B. Melanoma
C. Metastatic carcinoma
5. Postinflammatory
A. Argon laser coreoplasty
B. Exfoliation syndrome
C. Fibrinoid syndrome
D. Fungal endophthalmitis (see p. 223-225)
E. Iris neovascularization with pseudoexfoliation
F. Radiation
G. Surgery for retinal detachment
H. Uveitis, chronic
6. Vascular tufts at the pupillary margin
A. Cataract
B. Diabetes mellitus
C. Myotonic dystrophy syndrome (myotonia atrophica syndrome)
D. Ocular hypotony
E. Respiratory failure
Ulbig MR, et al. Anterior hyaloidal fibrovascular proliferation after extracapsular cataract
extraction in diabetic eye. Am J Ophthalmol 1993; 115:321-326.
HYPEREMIA OF IRIS (DILATATION OF PREEXISTING VESSELS OF THE

IRIS)
1. Corneal ulcer
2. Foreign body on the cornea
3. Injury, intraocular
*4. Iridocyclitis
*5. Iritis
6. Scleritis
*7. Uveitis
O'Brien CS. Ophthalmology: notes for students. Iowa City: Athens Press, 1930.
HETEROCHROMIA (DIFFERENCE OF COLOR BETWEEN TWO IRIDES)
1. Hypochromic heterochromia-abnormal eye with iris of lighter color than that of the
fellow eye
A. Anemia with unilateral iritis
B. Chédiak-Higashi syndrome (anomalous leukocytic inclusions with
constitutional stigmata)
*C. Congenital, sporadic, or familial
D. Conradi syndrome (epiphyseal congenital dysplasia)
E. Fuchs syndrome (I) (heterochromic cyclitis syndrome)
F. Gansslen syndrome (familial hemolytic icterus)
G. Glaucomatocyclitic crisis (Posner-Schlossman syndrome)
*H. Homer syndrome (cervical sympathetic paralysis syndrome)
I. Hypomelanosis of Ito syndrome (incontinentia pigmenti achromiens)
J. Infiltration of nonpigmented tumor into iris
*K. Iris atrophy (diffuse and unilateral), including that caused by trauma,
inflammation, or senility
L. Iris coloboma
M. Parry-Romberg syndrome (facial hemiatrophy)
N. Status dysraphicus syndrome (Bremer syndrome)
O. Tuberous sclerosis hypopigmented iris spot (Bourneville syndrome)
P. Waardenburg-Klein syndrome (embryonic fixation syndrome)
2. Hyperchromic heterochromia-abnormal eye with iris darker than that in the fellow eye
A. Anterior or posterior chamber hemorrhage, prolonged
B. Coloboma
*C. Congenital, sporadic, or familial
D. Embryonic fixation syndrome (Waardenburg-Klein syndrome)
E. Incontinentia pigmenti (Bloch-Sulzberger syndrome)
F. Iris abscess
G. Iris stromal cysts
H. Malignant melanoma of the iris or other pigmented tumors of the iris
I. Microcornea (see p. 252-253)
J. Monocular melanosis in which there are excess chromatophores in the stroma
of the iris (melanosis bulbi)
*K. Neovascular, such as rubeosis iridis or hyperemia of iris, unilateral (see p.
367 or p. 366)
L. Neurofibromatosis (von Recklinghausen syndrome)
*M. Nevi of iris
N. Perforating injuries or contusion of the globe occurring before the subject is
seven years of age
O. Retention of intraocular iron foreign body-siderosis
P. Severe contusion with hypertrophy of the superficial layers of the stroma of the
iris
Q. Status dysraphicus (Bremer syndrome)
3. Dark central pupillary margin, pale pigment around its circumference
A. Hereditary osteoonychodysplasia
B. Normal iris
Gutman I, et al. Hypopigmented iris spot. Ophthalmology 1982; 89:1155.
Lois N, et al. Primary iris stromal cysts. Ophthalmology 1998; 105:1317-1322.
Mann ES, et al. Iris coloboma with iris heterochromia. Arch Ophthalmol 2000; 118:1590-
1591.
1991.
Pfeiffer N, et al. Histological effects in the iris after 3 months of Latanoprost therapy.
Extracted Table Hyperchromic heterochromia (abnormal eye with iris of

darkercolor than fellow eye)
IRIS ATROPHY
1. Anterior segment ischemia syndrome

3. Chandler syndrome (iridocorneal endothelial syndrome)
4. Complication of light coagulation and beta radiation
5. Complication of retinal detachment operation
*6. Congenital-autosomal dominant
7. Crohn disease (granulomatous ileocolitis)
*8. Essential (progressive) atrophy
9. Glaucomatous atrophy
A. Acute-atrophy or iridoschisis
*B. Chronic-stromal and epithelial
10. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial syndrome)
11. Hilding syndrome (destructive iridocyclitis and multiple joint dislocations)
14. Iris nevus syndrome (Cogan-Reese syndrome)
15. Ischemia
A. Acute angle-closure glaucoma
B. Carotid-cavernous fistula
C. Hemoglobin sickle cell C disease
D. Occlusive artery disease
E. Orbital irritation
F. Surgery angle-closure glaucoma
*G. Trauma
16. Krause syndrome (congenital encephalo-ophthalmic dysplasia)
17. Neurogenic-tabes with stromal atrophy
*19. Old age
21. Posterior pigment layer is swollen and degenerated
B. Hurler syndrome (mucopolysaccharidoses I-H)
22. Postinflammatory-iritis because of diseases such as tuberculosis, syphilis (acquired
lues), herpes zoster, herpes simplex, smallpox, leprosy (Hansen disease), onchocerciasis
syndrome (river blindness), sporotrichosis
23. Shy-Magee-Drager syndrome (orthostatic hypotension syndrome)
24. Spontaneous progressive
A. Congenital hypoplasia iris stroma
B. Rieger syndrome (dysgenesis mesostromalis)
25. Takayasu syndrome (aortic arch syndrome, pulseless disease)
26. Use of quinine, chloramine, mustard gas
28. Xeroderma pigmentosa, including skin lesions
Rodrigues MM, et al. Clinical electron microscopic, and immunohistochemical study of

the corneal endothelium and Descemet's membrane in the iridocorneal endothelial
syndrome. Am J Ophthalmol 1986; 101:16-27.
Shields MB, et al. The essential iris atrophies. Am J Ophthalmol 1978; 85:749-769.
IRIDODONESIS (TREMULOUS IRIS)
*1. Aphakia following cataract extraction

*2. Dislocation of the lens (see p. 405)
3. Hydrophthalmos or buphthalmos (see p. 222-223)
4. Hypermature senile cataract
Filatov V, et al. Dislocation of the crystalline lens in a patient with Sturge Weber
Hornby SJ, et al. Visual acuity in children with coloboma. Ophthalmology 2000;
107:511-520.
Huggon IC, et al. Contractural arachnodactyly with mitral regurgitation and iridodonesis.
Arch Dis Child 1990; 65: 317-319.
TUMORS ARISING FROM PIGMENT EPITHELIUM OF IRIS
1. Hyperplasia
A. Primary (congenital)
(1) At pupillary margin
(2) At margins of colobomas
B. Acquired
(1) Region of sphincter-migrating epithelial cells appear in stroma as
clump cells (equivocal origin)
(2) Cells can reach anterior surface of iris and proliferate (velvety black in
appearance)
C. Secondary
(1) Intraocular inflammation-pigmented cells proliferate around the
papillary margin onto anterior iris surface
(2) Long-standing glaucoma
a. Proliferation around the pupillary margin onto the anterior iris
surface
b. Migration through stroma to anterior surface at collarette
*(3) Trauma (including operation-proliferation of pigment epithelium on
anterior surface of iris, across pupil, or on posterior surface of cornea
*(4) Drugs, including the following: demecarium, echothiophate,
edrophonium, isoflurophate, neostigmine, physostigmine, pilocarpine
often associated with cystic formation
2. Neoplasia
*A. Benign - well-differentiated epithelial cells, usually pigmented, often with
pseudoacinar arrangement and cysts; may have limited locally invasive properties
B. Malignant
(1) Carcinoma
(2) Local invasion, intraocular metastases
(3) Medulloepithelioma, embryonal type (diktyoma)
(4) Papillary cystadenoma
Morris PA, Henkind P. Neoplasms of the iris pigment epithelium. Am J Ophthalmol

1968; 66:31.
PIGMENTED LESIONS OF IRIS
1. Adenoma of iris
2. Anterior chamber intraocular lens and segmental uveal ectropion
3. Anterior staphyloma
4. Corneal or scleral perforation
*5. Cyst-congenital, spontaneous, or traumatic, including pigmentation
6. Ectopic lacrimal gland tissue
*7. Ectropion uvea
8. Epithelioma of the ciliary body
9. Exudative mass in the anterior chamber
10. Foreign body of iris, including iron with siderosis
11. Fuchs syndrome of heterochromic cyclitis with the darker normal iris considered to
contain a diffuse melanoma
12. Hemangioma of the iris with pigmentation because of hemorrhage
13. Hemosiderosis because of contusions with hyphema or injuries and disease in the
posterior portion of the eye with recurrent bleeding
14. Juvenile xanthogranuloma (nevoxanthoendothelioma)
15. Leiomyoma or leiomyosarcoma of the iris
16. Leukemic infiltrates and malignant lymphomas
17. Malignant melanoma of the iris
18. Metastatic carcinomas arising in the lung, breast, gastrointestinal tract, thyroid gland,
prostate gland, kidney, or testicle
19. Neurofibromatosis with increased pigmentation of the iris
*20. Nevi of the iris
21. Nodular thickening and scarring of the iris
22. Pigmentary glaucoma
23. Pigment epithelial tumors of the iris
24. Segmental melanosis oculi, including congenital melanosis
25. Stromal mass in the anterior chamber
26. Uveitis, such as that because of conglomerate tuberculous lesions of the stroma or
sarcoid involvement of the iris
27. Varix
Chang M, et al. Adenoma of the pigment epithelium of the ciliary body simulating a
malignant melanoma. Am J Ophthalmol 1979; 88:40-48.
Lois N, et al. Primary cysts of the iris pigment epithelium. Ophthalmology 1998;
105:1879-1885.
Shields JA, et al. Iris varix simulating an iris melanoma. Arch Ophthalmol 2000;
118:707-709.
Shields CL, et al. Differentiation of adenoma of the iris pigment epithelium from iris cyst
and melanoma. Am J Ophthalmol 1986; 100:678-681.
NONPIGMENTED LESIONS OF IRIS
1. Amelanotic melanoma
2. Atypical mycobacterial panophthalmitis
3. Endothelioma
4. Exudative mass in the anterior chamber
5. Fibrosarcoma
*6. Foreign body
7. Forward extension of diktyoma
8. Hemangioma of the iris
*9. Iris cyst
10. Iris lymphoma
11. Iris nodules
A. Ectodermal (Koeppe nodules)-pupillary margin and gray with ocular
inflammation
B. Mesodermal (Busacca nodules)-anterior surface of iris in collarette region
12. Juvenile xanthogranuloma-may be associated with diffuse infiltration of the iris
13. Lacrimal Gland Choristoma of the Iris
14. Leiomyoma or leiomyosarcoma of the iris
A. Lepromas of the iris
B. Leprotic pearl-minute white spots on the surface of iris
16. Metastatic carcinoma of the iris arising from the lungs, breast, gastrointestinal tract,
thyroid gland, prostate gland, kidney, or testicle
17. Neurofibroma and neuroglioma
18. Sarcoid nodules-multiple, discrete, irregularly distributed over the iris
19. Seeding of tumor, such as retinoblastoma, from the posterior segment
A. Gummas-solitary, large, avascular, white lesions
B. Papules (condylomas)-multiple, small, vascular, yellowish lesions
21. Teratoma
22. Tuberculosis
A. Acute miliary - small grayish yellow or reddish nodules
B. Hyalinized or fibrotic scar (Michel flecks)
C. Tuberculoma-white-gray lesion
Chan SM, et al. Iris lymphoma in a pediatric cardiac transplant recipient. Ophthalmology
2000; 107:1479-1482.
Imamura Y, et al. Gastric signet ring cell adenocarcinoma metastatic to the iris. Am J
Ophthalmol 2001; 131:379-381.
Kluppel M, et al. Lacrimal gland choristoma of the iris. Arch Ophthalmol 1999; 117:110-
111.
Rosenbaum PS, et al. Atypical mycobacterial panophthalmitis seen with iris nodules.
Arch Ophthalmol 1998; 116: 1524-1527.
Victor V, et al. Surgical excision of iris nodules in the management of sarcoid uveitis.
Ophthalmology 2001; 108: 1296-1299.
Ware GT, et al. Renal cell carcinoma with involvement of iris and conjunctiva. Am J
Ophthalmol 1999; 127, 4: 458-459.
CONDITIONS SIMULATING ANTERIOR UVEITIS OR IRITIS
1. Brushfield spots
2. Fuchs syndrome (II) (Stevens-Johnson syndrome)
3. Hereditary deep dystrophy of cornea
4. Hyalinized keratitic precipitate
5. Iridoschisis-splitting of iris
6. Juvenile xanthogranuloma of the iris (nevoxanthoendothelioma)
7. Malignant lymphomas or leukemia
9. Metastatic tumor arising from the lungs, breast, gastrointestinal tract, thyroid gland,
prostate gland, kidney, or testicle
10. Neurofibromas of the iris
11. Pigment floaters in the anterior chamber, especially after mydriasis
*12. Pseudoexfoliation of the lens capsule (glaucoma capsulare)
13. Reticulum cell sarcoma
14. Retinoblastoma
*16. Siderosis bulbi
Denslow GT, Kielar RA. Metastatic adenocarcinoma to the anterior uvea and increased
carcinoembryonic antigen levels. Am J Ophthalmol 1978; 85:378-382.
Schlaegel TF. Essentials of uveitis. Boston: Little, Brown, 1969.
SYNDROMES AND DISEASES ASSOCIATED WITH IRITIS
1. Actinomycosis
2. Amebiasis (entamoeba histolytica)
3. Amendola syndrome (Brasilian pemphigus)
4. Anderson-Warburg syndrome (congenital progressive oculoacousticocerebral
dysplasia)
5. Ankylosing spondylitis (von Beckterev-Strumpell syndrome)
6. Ascariasis
7. Aspergillosis
8. Beesting of the cornea
10. Blastomycosis
12. Candidiasis
13. Charlin syndrome (nasociliary nerve syndrome)
14. Chlamydia pneumoniae
15. Coccidioidomycosis
16. Cryptococcosis
17. Cysticercosis
18. Cytomegalic inclusion disease (cytomegalovirus)
19. Dengue fever
20. Endophthalmitis phacoanaphylactica
*21. Following laser iridectomy
22. Fuchs syndrome (heterochromic cyclitis syndrome)
23. Henoch-Schönlein purpura (anaphylactoid purpura)
24. Herbicide exposure-2, 4-dichlorophenoxyacetic acid
*25. Herpes simplex
*26. Herpes zoster
27. Histoplasmosis
28. Histiocytosis X (xanthomatous granuloma syndrome)
30. Leptospirosis (Weill disease)
31. Mucormycosis (phycomycosis)
32. Mustard gas injury
33. Mycoplasma pneumoniae
34. Nocardiosis
35. Onchocerciasis syndrome (Onchocerca volvulus)
36. Reiter syndrome (conjunctivourethrosynovial syndrome)
*39. Sarcoidosis syndrome (Schaumann syndrome)
40. Sporotrichosis
*41. Still disease (juvenile rheumatoid arthritis)
43. Toxoplasmosis (ocular toxoplasmosis)
44. Tuberculosis
45. Vaccinia
46. Vogt-Koyanagi-Harada syndrome (uveitis, vitiligo-alopecia-poliosis syndrome)
Solberg Y, et al. Ocular injury by mustard gas. Surv Ophthalmol 1997; 41:461-466.
Yamada I, et al. A child with iritis due to chlamydia pneumoniae infection. Journal of the
Japanese Association for Infectious Diseases 1994; 68:1543-1547.
IRITIS (ANTERIOR UVEITIS) IN CHILDREN
1. Anterior and posterior uveitis

A. Retinoblastoma
*B. Sarcoidosis syndrome (Schaumann syndrome)
C. Sympathetic ophthalmia
D. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis syndrome)
*2. Chronic cyclitis (peripheral uveitis)
3. Fuchs heterochromic cyclitis
4. Iridocyclitis
A. Acute tubulointerstitial nephritis and uveitis
B. Ankylosing spondylitis (von Beckterev-Strumpell syndrome)
C. Behçet syndrome (oculobuccogenital syndrome)
D. Juvenile xanthogranuloma
E. Kawasaki disease
F. Leukemia
G. Multiple sclerosis
H. Psoriatic arthropathy
I. Reiter syndrome
J. Retinal capillaritis
*K. Sarcoidosis syndrome (Schaumann syndrome)
L. Still syndrome (juvenile rheumatoid arthritis)
*M. Trauma
N. Ulcerative colitis and Crohn disease
O. Unknown
P. Viral-associated disease
5. Keratouveitis
*A. Herpes simplex
*B. Herpes zoster
Kimura SJ, Hogan MJ. Uveitis in children: analysis of cases. Trans Am Ophthalmol Soc
1964; 62:173.
Matsuo T, Matsuo N. Bilateral iridocyclitis with retinal capillaritis in juveniles.

Powell CJ, et al. Diffuse infiltrating retinoblastoma masquerading as a panuveitis.
Extracted Table Iritis (anterior uveitis) in children
NONGRANULOMATOUS UVEITIS
1. Physical insult
A. Endogenous
B. Exogenous
*2. Toxic insults
A. Autointoxication-ptomaines, protein split products, and so forth from food
poisoning
B. Bacterial endotoxins
C. Reticulum cell sarcoma of the brain
D. Toxins from disintegrating helminths
*E. Viral toxins
3. Immediate hypersensitive reaction
*A. Airborne allergens
acetazolamide cytarabine fluorometholone
adrenal cortex injection demecarium fluorouracil
aldosterone desoxycorticosterone fluprednisolone
amphotericin B dexamethasone gold Au 198
auranofin dibucaine gold sodium thiomalate
aurothioglucose dichlorphenamide gold sodium thiosulfate
aurothioglycanide diethylcarbamazine hyaluronate acid
bacille Calmette-Guerin diisopropyl hydrabamine
(BCG) vaccine flurophosphate hydroxypropylmethyl-
bacitracin (DFP) cellulose
beclomethasone diphtheria, pertussis, and ibuprofen
benoxinate tetanus (DPT) interleukin 3 and 6
betaxolol vaccine iodide and iodine
brimonidine disodium clodronate solutions and
bupivacaine disodium etidronate compounds
butacaine disodium pamidronate isoflurophate
chloramphenicol doxorubicin latanoprost
chloroprocaine dyclonine lidocaine
chlortetracycline echothiophate measle vaccine
chondroitin sulfate edrophonium medrysone
chymotrypsin emetine meprednisone
cidofovir epinephrine methazolamide
cobalt epinephrine methicillin
cocaine ethoxzolamide methylprednisolone
colistin etidocaine metipranolol
contraceptives floxuridine mitomycin
cortisone fludrocortisone neomycin
neostigmine propoxycaine sulfamethazine
paramethasone quinidine sulfamethizole
penicillin radioactive iodides sulfamethoxazole
penicillin reserpine sulfamethoxypyridazine
hydrocortisone rifampin sulfanilamide
penicillin V smallpox vaccine sulfapyridine
phenacaine sodium hyaluronate sulfasalazine
phenethicillin streptokinase sulfathiazole
physostigmine streptomycin sulfisoxazole
piperocaine sulfacetamide tetracaine
polymyxin B sulfachlorpyridazine tetracycline
potassium sulfacytine thiotepa
pralidoxime sulfadiazine triamcinolone
prednisolone sulfadimethoxine trifluoperazine
procaine sulfamerazine urokinase
proparacaine sulfameter
C. Foods
D. Protein antigens (anaphylaxis)
4. Delayed hypersensitive reaction
A. Bacterial antigens
B. Viral antigens
5. Doubtful entities-nongranulomatous uveitis
A. Amebiasis
B. Diabetic iritis
C. Gouty iritis
D. Heterochromic iridocyclitis
*E. Sarcoidosis syndrome (Schaumann syndrome)
F. Secondary to metabolic disease, such as biliary cirrhosis and systemic
xanthomatosis
G. Uveitis associated with collagen diseases
6. Mixed granulomatous and nongranulomatous
A. Lens-induced uvealis
*B. Peripheral uveitis
7. Human leukocyte antigen (HLA)-B27 associated diseases
A. Ankylosing spondylitis
B. Inflammatory bowel disease
C. Psoriasis
D. Reiter disease
8. Infections
A. Herpes zoster/herpes simplex
B. Lyme disease
C. Syphilis
Byles DB, et al. Anterior uveitis as a side effect of topical brimonidine. Am J Ophthalmol
2000; 130:287-291.
Chavez-de la Paz, et al. Anterior nongranulomatous uveitis after intravitreal HPMPC
(Cidofovir) for the treatment of cytomegalovirus retinitis. Ophthalmology 1997; 104:539-
544.
Moorthy RS, et al. Drug-induced uveitis. Surv Ophthalmol 1998; 42:557-570.
GRANULOMATOUS UVEITIS
1. Proven or probable etiology

A. Associated with nonpyogenic systemic infections
(1) Brucellosis (Brucella melitensis, B. abortus, B. suis)
(2) Leprosy (Mycobacterium leprae)
(3) Leptospirosis (Leptospira canicola, L. icterohaemorrhagiae, L.
pomona)
*(4) Syphilis (Treponema pallidum)
*(5) Tuberculosis (Mycobacterium tuberculosis)
B. Protozoan infections
(1) Amebiasis (Entamoeba coli, E. histolytica, Endolimax nana,
Acanthamoeba hartmannella)
(2) Toxoplasmosis (Toxoplasma gondii)
(3) Trypanosomiasis (Trypanosoma cruzi, T. gambiense)
C. Fungal infections
(1) Actinomycosis
(2) Aspergillosis
(3) Blastomycosis
(4) Candidiasis (moniliasis)
(5) Coccidioidomycosis
(6) Cryptococcosis (Cryptococcus neoformans or Torula histolytica)
*(7) Histoplasmosis (Histoplasma capsulatum)
(8) Mycomycosis (phycomycosis)
(9) Nocardiosis
(10) Sporotrichosis (Sporotrichum schenckii)
D. Helminth infestations
(1) Ascaridiosis (Ascaris lumbricoides)
(2) Cestodes
a. Cysticercosis (Cysticercus cellulosae)
b. Taeniasis (Taenia echinococcus)
(3) Diptera larvae (exogenous)
(4) Nematodes
a. Ancylostomiasis (Toxocara canis, Ancylostoma duodenale,
Ancylostoma caninum, Necator americanus)
b. Onchocerciasis (Onchocerca volvulus)
E. Viral-herpes zoster
2. Recognized clinical and histopathologic entity, of unknown cause
*A. Multiple sclerosis
*B. Sarcoidosis syndrome (Schaumann syndrome)
C. Sympathetic ophthalmia
3. Nonspecific granulomatous uveitis of unknown cause, including granulomatous
ileocolitis
4. Mixed granulomatous and nongranulomatous
A. Lens-induced uveitis
B. Peripheral uveitis
5. Viral uveitis
A. Proven or probable
(1) Cytomegalic inclusion disease
(2) Herpes simplex
(3) Herpes zoster
(4) Vaccinia
B. Suspected
(1) Behçet syndrome (oculobuccogenital syndrome)
(2) Retinal capillaritis
(3) Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis
syndrome)
6. Histiocytosis X (includes eosinophilic granuloma, Hand-Schüller-Christian disease and
Letterer-Siwe disease)
7. Following treatment of a choroidal melanoma with proton-beam irradiation
Cho AS, et al. Ocular involvement in patients with post-transplant lymphoproliferative

disorder. Arch Ophthalmol 2001; 119: 183-185.
Matsuo T, Matsuo N. Bilateral iridocyclitis with retinal capillaritis in juveniles.

Schwab IR. Herpes zoster sine herpete. Ophthalmology 1997; 104:1421-1425.
PIGMENTED CILIARY BODY LESIONS
* 1. Ciliary body cyst

2. Diffuse iris melanotic lesion
*3. Drugs including the following:
adrenal cortex injection edrophonium neostigmine
aldosterone epinephrine paramethasone
betamethasone fludrocortisone physostigmine
cortisone fluprednisolone pilocarpine
demecarium hydrocortisone prednisolone
desoxycorticosterone isoflurophate prednisone
dexamethasone meprednisone triamcinolone
echothiophate methylprednisolone
5. Melanocytoma of ciliary body
6. Peripheral uveal detachment
7. Posttraumatic pigmentary migration
Biswas J, et al. 7 diffuse melanotic lesion of the iris as a presenting feature of ciliary
body melanocytoma: report of a case and review of the literature. Surv Ophthalmol 1998;
42:378-383.
Lois N, et al. Cavitary melanoma of the ciliary body. Ophthalmology 1998; 105:1091-
1098.
NEUROEPITHELIAL TUMORS OF CILIARY BODY
1. Congenital
A. Glioneuroma
B. Medulloepithelioma
*(1) Benign
(2) Malignant
C. Teratoid medulloepithelioma
(1) Benign
(2) Malignant
2. Acquired
A. Adenocarcinoma
*(1) Papillary
(2) Pleomorphic
(3) Solid
B. Adenoma
*(1) Papillary
(2) Pleomorphic
(3) Solid
C. Mesectodermal leiomyoma
D. Pseudoadenomatous hyperplasia
Shields JA, et al. Observations on seven cases of intraocular leiomyoma. Arch

Ophthalmol 1994; 112:521-528.
Shields JA, et al. Natural causes and histopathologic findings of lacrimal gland
chorestoma of the iris and ciliary body. Am J Ophthalmol 1995; 119:219-224.
INTERNAL OPHTHALMOPLEGIA
Internal ophthalmoplegia is characterized by paresis of ciliary body with loss of
power of accommodation and pupil dilatation because of lesions of ciliary ganglion.
1. Acute porphyria-frequently bilateral

*2. Adie syndrome (myotonic pupil)
3. Aneurysm of the posterior communicating artery at its junction with the internal
carotid-unilateral
4. Congenital-rare
*5. Cycloplegic ocular medication-most common
6. During acute illness-transient
7. During blepharoplasty-transient
8. Fisher syndrome (ophthalmoplegia-ataxia-areflexia syndrome)
9. Foramen lacerum syndrome (aneurysm of internal carotid artery)
*12. Increased intracranial pressure
13. Infections, including chickenpox, measles, diphtheria, syphilis, scarlet fever,
pertussis, smallpox, influenza, herpes zoster, botulism, sinusitis, and viral hepatitis
14. Lubarsch-Pick syndrome (amyloidosis)
15. May be early lesion of acute or chronic ophthalmoplegia
16. Metastatic tumors of choroid
17. Nasopharyngeal carcinoma-early
18. Nothnagel syndrome (ophthalmoplegia-cerebellar ataxia syndrome)
19. Partial seizures
20. Retrobulbar injections of alcohol
21. Transscleral diathermy
22. Trauma to eye or orbit
Perlman JP, Conn H. Transient internal ophthalmoplegia during blepharoplasty: a report

of three cases. Ophthal Plast Reconstr Surg 1991;7:141-143.
Rosenberg ML, Jabbari B. Miosis and internal ophthalmoplegia as a manifestation of

partial seizures. Neurology 1991; 41:737-739.
13
Lens
CONTENTS
Anterior subcapsular cataract 386
Nuclear cataracts 391
Lamellar cataracts 391
Punctate cataracts 392
Posterior subcapsular cataract 393
Iridescent crystalline deposits in lens 399
Oil droplet in lens 399
Lenticonus and lentiglobus 399
Lens absorption 400
Exfoliation of lens capsule 400
Microphakia or spherophakia or microspherophakia 400
Dislocated lens 401
Aphakia 405
Equatorial lens pigmentation 405
Unilateral cataracts 405
Lenticular disease associated with corneal problems 405
Drugs associated with cataracts 409
Syndromes and diseases associated with cataracts 410
Lenticulocorneal adherence 416
Spasm of accommodation 416
Paresis of accommodation 417
ANTERIOR SUBCAPSULAR CATARACT
1. Acrodermatitis chronica atrophicans

2. Addison syndrome (adrenal cortical insufficiency)
3. Albinism
4. Allopurinol therapy
6. Amiodarone usage
7. Andogsky syndrome (dermatogenous cataract)
8. Aniridia
9. Anterior chamber air
10. Atopic (eczema cataract)
11. Beesting of cornea
13. Chlorpromazine therapy
14. Chromosomal 3; 18 translocation
15. Comedo cataract
16. Coughing
17. Cryotherapy
18. Electric cataract
*19. Diabetes mellitus (Willis disease)
20. Facial paralysis (partial)
21. Frenkel syndrome (ocular contusion syndrome)
22. Goldscheider syndrome (epidermolysis bullosa)
23. Gyrate atrophy (ornithine ketoacid aminotransferase deficiency)
24. Head-banging (chronic)
25. Hemifacial microsomia syndrome (François- Haustrate syndrome)
26. Hypermature cataract with other changes
28. Idiopathic-l0% of normal population
29. Intraocular copper and iron
30. Isotretinoin
31. Jadassohn-Lewandowsky syndrome (epidermolysis bullosa)
33. Marinesco-Sjögren syndrome (oligophrenia syndrome)
35. Naphthalene ingestion
36. Neodymium:yttrium-aluminum-garnet (Nd:YAG)
37. Neurodermatitis
38. Pemphigus foliaceous (Cazenave disease)
39. Phenothiazine therapy
40. Phospholine iodide use
41. Pseudohypoparathyroidism
42. Reese-Ellsworth syndrome (anterior chamber cleavage syndrome)
43. Rothmund syndrome (telangiectasia-pigmentation-cataract syndrome)
44. Scaphocephaly
45. Schizophrenia
46. Thorazine ingestion
*47. Trauma, such as contusion
48. Tyrosinosis
49. Vitrectomy for diabetic retinopathy
52. Zinc chloride (concentrated)
McCarty CA, et al. Schizophrenia, psychotropic medication, and cataract. Ophthalmology

1999; 106:683-687.
Roy FH. Ocular syndromes and systemic disease, 3rd ed. Philadelphia: Lippincott
Zadok D, Chayet A. Lens opacity after neodymium: YAG laser iridectomy for phakic
intraocular lens implantation. J Cataract Refract Surg 1999; 25:592-593.
Extracted Table Anterior subcapsular cataract
NUCLEAR CATARACTS
1. Alcohol
3. Associated with photocoagulation, such as argon laser use
4. Capsular exfoliation syndrome
5. Congenital dysplasia
6. Conradi syndrome (multiple epiphyseal congenital dysplasia)
7. Coppock cataract, discoid cataract, zonular cataract -autosomal dominant
8. Hyperbaric oxygen therapy
9. Maple-syrup urine disease (branched-chain ketoaciduria)
10. Matsouka syndrome (oculocerebroarticuloskeletal syndrome)
11. Micro syndrome
12. Nuclear diffuse nonprogressive cataract-autosomal dominant, rarely recessive
*13. Nuclear sclerosis
*A. Pars plana vitrectomy for macular pucker
B. Smoking
14. Nuclear total cataract-autosomal dominant, rarely recessive
15. Paradichlorobenzene (mothballs)
16. Perforating injuries
17. Roy syndrome II-nuclear cataract associated with smoking
*18. Rubella syndrome (German measles)
19. Siemen syndrome (hereditary ectodermal dysplasia syndrome)
20. von Gierke disease (glucose-phosphate deficiency)
Klein RE, et al. Cigarette smoking and lens opacities-the Beaver Dam Eye Study. Am J
Prey Med 1993; 9:27-30.
Leung ATS, et al. Chlorpromazine-induced refractile corneal deposits and cataract. Arch
Opthalmol 1999; 117: 1662-1663.
Seddon J, Fong D. The association between cigarette smoking and ocular diseases. Surv
Ophthalmol 1998; 42: 535-547.
LAMELLAR (STELLATE, ZONULAR, CORTICAL, CORONARY)

CATARACTS
1. Alcohol
2. Aniridia
3. Argon laser
4. Autosomal-dominant congenital cataract
5. Congenital zonular cataract
6. Cortical cataract and congenital ichthyosis
7. Dermochonoral corneal dystrophy
9. Females
10. Galactokinase deficiency (von Reuss syndrome)
11. Hagberg-Santavuori (neuronal ceroid-lipofuscinoses)
12. Hypertension
13. Hypoglycemia
15. Iritis
16. Leiomyoma
17. Mannosidosis
18. Marfan syndrome (dolichostenomelia-arachnodactyly-hyperchondroplasia-dystrophia
mesodermalis congenital
19. Marshall syndrome (atypical ectodermal dysplasia)
21. Neurofibromatosis 2 (central neurofibromatosis)
22. Nieden syndrome (telangiectasia cataract syndrome)
23. Nonwhites
24. Obesity (abdominal)
25, Organic nitrate explosives
26. Passow syndrome (status dysraphicus syndrome)
27. Riboflavin deficiency (Stannus cerebellar syndrome)
28. Roy syndrome (cataract associated with smoking)
29. Sunlight
30. Tetany cataract (hypoparathyroidism)
31. Ultraviolet-B light
33. Van Bogaert-Scherer-Epstein syndrome (primary hyperlipidemia)
34. Van der Hoeve syndrome (osteogenesis imperfecta)
35. Zonular cataract and nystagmus-X-linked
Leske MC, et al. Diabetes, hypertension, and central obesity as cataract risk factors in a
black population. Ophthalmology 1999; 106:35-41.
Roy FH. Cigarette smoking and the risk of cataract [Letter]. JAMA 1993; 269:748.
Younger CR, et al. Lens opacifications detected by slitlamp biomicroscopy are associated
with exposure to organic nitrate explosives. Arch Ophthalmol 2000; 118:1653-1657.
PUNCTATE CATARACTS (NUMEROUS SMALL OPACITIES)

2. Argon laser
3. Autosomal dominant vitreoretinochoroidopathy (ADVIRC)
6. Galactokinase deficiency (von Reuss syndrome)
7. Hypercalcemia (adult)
8. Hyperprolactinemia
9. Incontinentia pigmenti achromians
11. Rothmund syndrome (telangiectasia-pigmentation-cataract syndrome)
12. Supravalvular aortic stenosis (Williams-Beuren syndrome)
Costagliola C. Hyperprolactinemia and lens opacities. Ann Ophthal 1992; 24:418-419.
Drack AV, et al. Transient punctate lenticular opacities as a complication of Argon laser
photocoagulation in an infant with retinopathy of prematurity. Am J Ophthalmol 1992;
113:583.
POSTERIOR SUBCAPSULAR CATARACT
1. Complicated cataract
A. Anterior segment involvement, such as that because of the following:
(1) Acute and chronic corneal ulcer
*(2) Iridocyclitis
*(3) Chronic anterior uveitis
(4) Acute or chronic glaucoma
B. Posterior segment involvement such as that because of the following:
*(1) Chronic posterior uveal inflammation
(2) Long-standing retinal detachment
(3) High myopia
(4) Hereditary retinal lesions, including retinitis pigmentosa
(5) Persistent hyperplastic primary vitreous
2. Congenital posterior polar lens changes
A. Spurious posterior capsular cataract (Mittendorf dot)
B. Posterior polar cataract-persistent fibrovascular sheath of lens with or without
secondary cataract
C. Posterior lenticonus
3. Aberfeld syndrome (ocular and facial abnormalities syndrome)
4. Acrodermatitis chronic a atrophicans
5. Alcoholism
6. Aniridia
8. Aspergillosis
11. Buerger disease (thromboangiitis obliterans)
13. Carotid artery syndrome
14. Chromosome partial deletion (short-arm) syndrome
15. Congenital amaurosis of Leber (Leber congenital amaurosis)
16. Cushing syndrome
18. Drugs, including dinitrophenol busulfan (Myleran), triparanol (MER-29), PUVA
(psoralen plus ultraviolet light of A-wave length), allopurinol, indapamide, megestrol
acetate, and phenothiazine usage
21. Fabry disease (glycosphingolipid lipidosis)
22. Familial hypogonadism syndrome
24. Fuchs syndrome
25. Glassblowers (heat) cataract
27. Hagberg-Santavuori syndrome (neuronal ceroid-lipofuscinoses)
28. Hair dye
29. Hand-Schüller-Christian syndrome (xanthomatous granuloma syndrome)
30. Harada syndrome (uveitis-vitiligo-alopecia-poliosis syndrome)
31. Heerfordt syndrome (uveoparotitis)
32. Hemochromatosis
33. Herpes simplex
34. Hodgkin disease
35. Hypertension
37. Ionizing radiation, such as that encountered in x-ray, radium, or neutron therapy
38. Jacobsen-Brodwall syndrome
40. Kyrle disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans)
41. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-adipo-
sogenital)
44. Leukemia
45. Lightning induced
46. Malaria
47. Meckel syndrome (dysencephalia splanchnocystic syndrome)
49. Neurodermatitis (lichen simplex chronicus)
50. Neurofibromatosis 1 (von Recklinghausen syndrome)
52. Ocular trauma (blunt)
53. Oculootoororenoerythropoietic disease
54. O'Donnell-Pappas syndrome (foveal hypoplasia and presenile cataract-autosomal
dominant)
57. Pernicious anemia syndrome
59. Posterior polar cataract-autosomal dominant
62. Renal transplantation
63. Retinitis pigmentosa-deafness-ataxia syndrome
64. Roy syndrome I (unilateral cataract associated with smoking)
*65. Senile posterior cortical cataract
67. Silicone oil (intraocular)
*68. Steroid usage (topical or systemic)
69. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)
70. Still disease (juvenile rheumatoid arthritis)
71. Toxocariasis (Nematode ophthalmia syndrome)
72. Trisomy (Patau syndrome)
73. Tuomaala-Haapanen syndrome (similar to pseudohypoparathyroidism)
75. Ultraviolet-B light
76. Uric acid (increased serum levels)
77. Vitrectomy for diabetic retinopathy
78. Weil disease (leptospirosis)
80. Yersiniosis
Seddon J, Pong D. The association between cigarette smoking and ocular diseases. Surv
Ophthalmol 1998; 42: 535-547.
Tong JT, Bateman JB. Selective B-wave reduction with congenital cataract in
neurofibromatosis-2. Ophthalmology 1999; 106: 1681-1683.
Extracted Table Posterior subcapsular cataract
IRIDESCENT CRYSTALLINE DEPOSITS IN LENS
1. Idiopathic
2. Hypothyroid (cretinism)
3. Hypocalcemia
A. Postoperative-removal of thyroid and accidental parathyroid removal
B. Idiopathic hypoparathyroidism
C. Pseudohypoparathyroidism (hypoparathyroid cretinism) or with
hyperphosphatemia (Albright disease)
D. Pseudopseudohypoparathyroidism (brachymetacarpal dwarfism)
acetophenazine gold Au 198 prochlorperazine
amiodarone gold sodium thiomalate promazine
auranofin gold sodium thiosulfate promethazine
aurothioglucose mercuric oxide propiomazine
aurothioglycanide mesoridazine silver nitrate
butaperazine methdilazine silver protein
carphenazine methotrimeprazine thiethylperazine
chlorpromazine mild silver protein thiopropazate
chlorprothixene perazine thioridazine
colloidal silver pericyazine thiothixene
diazepam (?) perphenazine trifluoperazine
diethazine phenylmercuric acetate triflupromazine
ethopropazine phenylmercuric nitrate trimeprazine
fluphenazine piperacetazine
6. Cataract (coralliform and aculeiform) usually autosomal dominant; sometimes

recessive
OIL DROPLET IN LENS
1. Anterior displacement of lens

*2. Galactosemia-transferase deficiency (von Reuss syndrome)
3. Lenticonus
Bellows JG, Bellows RT. Displacement of the lens. In: Bellows JG, ed. Cataract and
abnormalities of the lens. New York: Grune & Stratton, 1975:277.
Beutler E, et al. Galactokinase deficiency as cause of cataract. N Engl J Med 1973;

288:1203-1206.
LENTICONUS (CONICAL LENS SURFACE PROTUBERANCE) AND

LENTIGLOBUS (GLOBULAR LENS SURFACE PROTUBERANCE)
1. Anterior-rare and usually bilateral

*A. Alport syndrome (hereditary nephritis)
B. Spina bifida
C. Waardenburg syndrome (embryonic fixation syndrome)
2. Posterior-more common and often unilateral
A. Associated with persistent hyperplastic primary vitreous
B. Associated with remnants of hyaloid artery
C. Familial posterior lenticonus and microcornea
*D. Lowe syndrome (oculocerebrorenal syndrome)
E. Trauma
Bleik JH, et al. Familial posterior lenticonus and microcornea. Arch Ophthalmol 1992;
110:1208.
Junk AK, et al. Bilateral anterior lenticonus. Arch Ophthalmol 2000; 118:895-897.
Tripathi RC, et al. Pathogenesis of cataracts in patients with Lowe's syndrome.

Ophthalmology 1986; 93: 1046-1051.
LENS ABSORPTION
3. Surgical trauma as discission
4. Trauma, blunt or penetrating
EXFOLIATION OF LENS CAPSULE

In this condition, superficial layers of the lens capsule split off and float in
aqueous as a fine membrane.
*1. Senile exfoliation

2. Toxic exfoliation
A. Atrophic eyes
B. Prolonged iridocyclitis
C. Lodgment of metallic foreign body, such as iron or copper
3. Traumatic
A. Perforating injury
B. Contusions with suspensory ligament separated from a dislocated lens
4. Heat exposure, such as that experienced by glassblowers
Duke-Elder S. System of ophthalmology. Vol XI. St. Louis: CV Mosby, 1969.
Meades K, Versace P. True exfoliation of the lens capsule. Aust N Z J Ophthalmol 1992;
20:347-348.
MICROPHAKIA OR SPHEROPHAKIA OR MICROSPHEROPHAKIA (SMALL

LENS OR HIGHLY SPHERIC LENS)
1. Achard syndrome (Marfan syndrome with dysostosis mandibulofacialis)

3. Familial anomaly
5. Hyperlysinemia
6. Lenticular myopia as recessive inheritance trait
7. Little syndrome (hereditary osteoorcychodysplasia)
8. Lowe syndrome (renal rickets)
*9. Marchesani syndrome (brachymorphy with spherophakia) (Weill-Marchesani
syndrome)
10. Marfan syndrome (dolichostenomelia-arachnodactyly hyperchondroplasia
dystrophica mesodermalis congenita)
11. Peter anomaly (anterior chamber cleavage syndrome)
12. Reticular dystrophy of the retinal pigment epithelium
Sorsby A. Ophthalmic genetics, 2nd ed. New York: Appleton-Century-Crofts, 1970.

DISLOCATED LENS
1. Achard syndrome (Marfan with dysostosis)

2. Adenoma of the nonpigmented epithelium of the ciliary body
3. Apert syndrome (sphenoacrocraniosyndactyly)
4. Ascariasis
*A. Aniridia
B. Coloboma of iris and choroid
*C. Congenital glaucoma
D. Ectopia lentis et pupillae
E. Focal dermal hypoplasia (Goltz syndrome)
F. High myopia
G. Isolated lens dislocation (up)
H. Megalocornea
I. Microcornea
J. Microspherophakia with hernia
K. Pseudoxanthoma
L. Retinitis pigmentosa
6. Autosomal recessive or dominant abnormality without other defects, except usually
ectopic pupils
8. Crouzon disease
9, Cryptophthalmia syndrome (cryptophthalmos-syndactyly syndrome)
10. Dwarfism, genetic type
11. Ehlers-Danlos syndrome (cutis hyperelastica)
12. Gillum-Anderson syndrome (blepharoptosis, myopia, ectopia lentis)
13. Gorlin-Goltz syndrome
15. Hereditary ectodermal dysplasia syndrome
*16. Homocystinuria-usually downward displacement of lens
17. Hyperlysinemia
18. Kniest syndrome
19. Late-onset localized junctional epidermolysis bullosa and mental retardation
21. Marchesani syndrome (brachymorphy with spherophakia)(Weill-Marchesani
syndrome)
*22. Marfan syndrome (dolichostenomelia-arachnodactyly-hyperchondroplasia
dystrophia mesodermalis congenita)-usually superior displacement of lens
23. Molybdenum cofactor deficiency (combined deficiency of sulfite oxidase and
xanthine dehydrogenase)
24. Oculodental syndrome (Peters syndrome)
25. Pfaundler syndrome
26. Pierre Robin syndrome
27. Pseudoexfoliation (exfoliation syndrome)
28. Recession of anterior chamber angle
29. Refsum syndrome
30. Retinal disinsertion syndrome
31. Retinoblastoma
33. Spherophakia (see p. 400)
34. Spontaneous (degenerative)
35. Sprengel deformity
36. Sturge-Weber syndrome
37. Sulfite oxidase deficiency
38. Surgical accidents (iatrogenic)
39. Syphilis (lues)
40. Trauma as in Frenkel syndrome (ocular contusion syndrome), beesting, and following
YAG laser
41. Treacher Collins syndrome (mandibulofacial dysostosis)
42. Uveitis
43. Wildervanck syndrome (cervicooculoacoustic syndrome)
Byrnes BA, et al. Retinoblastoma presenting with spontaneous hyphema and dislocated
lens. J Pediatr Ophthalmol Strabismus 1993; 30:334-336.
Edwards MC, et al. Isolated sulfite oxidase deficiency. Ophthalmology 1999; 106:1957-
1961.
Melamed S, et al. Neodymium: YAG laser iridotomy as a possible contribution to lens

dislocation. Ann Ophthalmol 1986; 18:281-282.
Extracted Table Dislocated lens
APHAKIA (ABSENCE OF LENS IN USUAL POSITION BEHIND THE IRIS)
1. Congenital absence of lens-rare

2. Dislocation of the lens into vitreous cavity, anterior chamber, or subconjunctival area
3. Following cataract extraction
4. Gradual absorption of the lens
Hanna C, et al. Extraocular traumatic luxation of the lens. J Ark Med Soc 1969; 66:210.
EQUATORIAL LENS PIGMENTATION

1. Associated with myopia and retinal detachment
2. Congenital malformation
3. Pigmentary glaucoma
4. Uveitis
Delaney WV Jr. Equatorial lens pigmentation, myopia, and retinal detachment. Am J

Ophthalmol 1975; 79:194-205.
UNILATERAL CATARACTS
1. Argon laser treatment

*2. Trauma
3. Complicated cataract
A. Anterior segment involvement, such as that because of the following:
(1) Acute and chronic corneal ulcer
*(2) Iridocyclitis
(3) Chronic anterior uveitis
(4) Acute or chronic glaucoma
B. Posterior segment involvement, such as that because of the following:
(1) Chronic posterior uveal inflammation
(2) Long-standing retinal detachment
(3) High myopia
(4) Hereditary retinal lesions
(5) Persistent hyperplastic primary vitreous
4. Roy syndrome I (unilateral cataract associated with smoking)
5. Congenital posterior polar lens changes
6. Glaucomatocyclitic crisis (Posner-Schlossman syndrome)
7. Conditions that give bilateral manifestations with earlier onset in one eye
Jarrett WH. Dislocation of the lens. Arch Ophthalmol 1967; 78:289.
Roy FH. Cigarette smoking and the risk of cataract [Letter]. JAMA 1993; 269:748.
LENTICULAR DISEASE ASSOCIATED WITH CORNEAL PROBLEMS
1. Aberfeld syndrome (ocular and facial abnormalities syndrome)-cataracts, microcornea

2. Acrodermatitis chronic a atrophicans - keratomalacia, corneal opacification, cataracts
3. Addison syndrome (idiopathic hypoparathyroidism)- keratoconjunctivitis, corneal
ulcers, keratitic moniliasis, cataracts
4. Alport syndrome (hereditary nephritis)-anterior lenticonus, posterior polymorphous
corneal dystrophy
*5. Amiodarone - corneal deposits, anterior subcapsular cataracts
6. Amyloidosis - amyloid deposits of cornea, corneal dystrophy, pseudopodia lentis
opacification and lenticular destruction with a mass visible behind the lens
8. Andogsky syndrome (atopic cataract syndrome)-atopic keratoconjunctivitis,
keratoconus, uveitis, subcapsular cataract
9. Aniridia-microcornea and subluxated lenses
10. Anterior-chamber cleavage syndrome (Reese-Ellsworth syndrome)-corneal opacities,
anterior pole cataract
11. Anterior segment ischemia syndrome-corneal edema, corneal ulceration, cataract
12. Apert syndrome (absent digits cranial defects syndrome)-exposure keratitis, cataracts,
ectopia lentis
13. Arteriovenous fistula (arteriovenous aneurysm)-bullous keratopathy, cataract
14. Aspergillosis-corneal ulcer, keratitis, cataract
*15. Atopic dermatitis (atopic eczema, Besnier prurigo)-keratoconjunctivitis,
keratoconus, cataract
16. Autosomal dominant-cataracts and microcornea
17. Avitaminosis C (scurvy)-keratitis, corneal ulcer, cataract
18. Chalcosis (intraocular copper containing foreign body) deposits in Descemet
membrane and anterior lens capsule
19. Chickenpox (varicella)-corneal ulcer, corneal opacity, keratitis, cataract
20. Chlorpromazine - corneal and lens opacities
21. Cholera-keratomalacia, cataract
22. Chromosome partial deletion (short-arm) syndrome-cataracts, corneal opacities
23. Chrysiasis (gold)-corneal and lens deposits
24. Cockayne syndrome (Mickey Mouse syndrome)-cataracts, band keratopathy, corneal
dystrophy
25. Congenital spherocytic anemia (congenital hemolytic jaundice)-congenital cataract,
ring-shaped pigmentary corneal deposits
26. Crouzon syndrome (Parrot-head syndrome)-exposure keratitis, cataract, corneal
dystrophy
27. Cryptophthalmia syndrome (cryptophthalmos-syndactyly syndrome)-cornea
differentiated from sclera, lens absence to hypoplasia, dislocation, and calcification
28. Cytomegalic inclusion disease (cytomegalovirus)-cataract, corneal opacities
29. Darier-White syndrome (keratosis follicularis)-keratosis, corneal subepithelial
infiltrations, corneal ulceration, cataract
30. Dermatitis herpetiformis (Duhring-Broca disease)-corneal vascularization, cataract
31. Dermochondral corneal dystrophy (of François)-cataract, corneal dystrophy
32. Diabetes mellitus-cataract, corneal edema secondary to rubeosis
33. Diphtheria-keratitis, corneal ulcer, cataract
34. Down syndrome (trisomy 21)-lens opacities, keratoconus
35. Ehler-Danlos syndrome (fibrodysplasia elastica generalisata)-microcornea,
keratoconus, lens subluxation
36. Electrical injury-corneal perforation, necrosis of cornea, anterior or posterior
subcapsular cataracts
37. Endothelial dystrophy and anterior polar cataract (Dohlman)
38. Exfoliation syndrome (capsular exfoliation syndrome)-cataract, dislocated lens,
corneal dystrophy, lens capsule exfoliation
39. Fabry disease (glycosphingolipid lipidosis)-cataract, corneal dystrophy
40. Folling syndrome (phenylketonuria)-corneal opacities, cataracts
41. Fuchs syndrome (I) (heterochromic cyclitis syndrome)-secondary cataract, edematous
corneal epithelium
42. Goldscheider syndrome (epidermolysis bullosa)-bullous keratitis, corneal
subepithelial blisters to corneal perforation, cataract
43. Gorlin-Goltz syndrome (multiple basal cell nevi syndrome)-cataract, corneal leukoma
44. Grönblad-Strandberg syndrome (elastorrhexis)-keratoconus, cataract, subluxation of
lens
45. Hallermann-Streiff syndrome (oculomandibulofacial dyscephaly)-cataracts,
microcornea
46. Hanhart syndrome (recessive keratosis palmoplantaris)-dendritic corneal lesions,
keratitis, corneal haze, corneal neovascularization, cataract
47. Heerfordt syndrome (uveoparotid fever)-band keratopathy, keratoconjunctivitis,
cataract
48. Hereditary ectodermal dysplasia syndrome (Siemens syndrome)-keratosis, corneal
erosions, corneal dystrophy, cataract, lens luxation
49. Herpes simplex-keratitis, corneal ulcer, cataracts
50. Herpes zoster-keratitis, corneal ulcer, cataract
51. Histiocytosis X (Hand-Schüller-Christian syndrome)-pannus, bullous keratopathy,
corneal ulcer, cataract
52. Hodgkin disease-keratitis, cataract
53. Homocystinuria syndrome-dislocated lens, cataract, keratitis
54. Hutchinson-Gilford syndrome (progeria)-cataract, microcornea
55. Hydatid cyst (echinococcosis)-keratitis, corneal abscess, cataract
56. Hypervitaminosis D-band keratopathy, cataract
57. Hypoparathyroidism-keratitis, cataract
58. Hypophosphatasia (phosphoethanolaminuria)-cataract, corneal subepithelial
calcifications
59. Influenza-keratitis, cataract
60. Jadassohn-type anetodermal-keratoconus, cataract
61. Jadassohn-Lewandowsky syndrome (pachyonychia congenita)-corneal dyskeratosis,
cataract
*62. Juvenile rheumatoid arthritis (Still disease)-band keratopathy, cataract
63. Kussmaul disease (periarteritis nodosa)-corneal ulcer, cataract
64. Kyrle disease (hyperkeratosis penetrans)-subcapsular cataracts, subepithelial corneal
opacities
65. Leber congenital amaurosis-cataracts, keratoconus
66. Leri syndrome (carpal tunnel syndrome)-corneal clouding, cataract
67. Listerellosis (listeriosis)-keratitis, corneal abscess and ulcer, cataract
68. Little syndrome (nail-patella syndrome)-microcornea, keratoconus, cataract
*69. Lowe syndrome (oculocerebrorenal syndrome)-cloudy cornea, cataracts,
megalocornea, corneal dystrophy
70. Malaria-keratitis, cataract
71. Marchesani syndrome (brachymorphy with spherophakia)-lenticular myopia, ectopia
lentis, megalocornea, corneal opacity
72. Marfan syndrome (arachnodactyly-dystrophia-mesodermalis congenita)-lens
dislocation, cataract, megalocornea, lenticular myopia
73. Matsoukas syndrome (oculocerebroarticuloskeletal syndromes)-cataract, corneal
sclerosis
74. Meckel syndrome (dysencephalia splanchnocystic syndrome)-sclerocornea,
microcornea, cataract
75. Micro syndrome (autosomal recessive microcephaly, microcornea, congenital
cataract, mental retardation, optic atrophy, and hypogenitalism)
76. Morbilli (rubeola, measles)-corneal ulcer, cataract
77. Mucolipidosis IV (ML IV)-corneal clouding, cataract
78. Myotonic dystrophy (Curschmann-Steinert syndrome)-lens opacity, cornea-epithelial
dystrophy
79. Nematode ophthalmia syndrome (toxocariasis)-cataract, larvae present in the cornea
80. Neurodermatitis (lichen simplex chronicus)-keratoconjunctivitis, atopic cataracts,
keratoconus
81. Neurofibromatosis 1
82. Neurofibromatosis 2
83. Ocular toxoplasmosis (toxoplasmosis)-keratitis, cataract
84. Oculodental syndrome (Peter syndrome)-macrocornea, opacities of the corneal
margin, ectopic lentis, corneoscleral staphyloma
85. O'Donnell-Pappas syndrome-presenile cataract, peripheral corneal pannus
86. Paget syndrome (osteitis deformans)-corneal ring opacities, cataract
87. Passow syndrome (status dysraphicus syndrome)-neuroparalytic keratitis, zonular
cataract
88. Pemphigus foliaceus (Cazenave disease)-pannus, corneal infiltration, cataract
89. Peter anomaly (anterior chamber cleavage syndrome-lens apposition to leukoma
90. Pigmentary ocular dispersion syndrome (pigmentary glaucoma)-Krukenberg spindle,
equatorial pigmentation of lens capsule
91. Pseudohypoparathyroidism (Seabright-Bantan syndrome)-punctate cataracts, keratitis
92. Radiation-corneal ulcer, punctate keratitis, cataracts, exfoliation of lens capsule
93. Refsum syndrome (phytanic acid oxidase deficiency)-corneal opacities, cataracts
94. Relapsing polychondritis-corneal ulcer, cataracts, keratoconjunctivitis sicca
95. Retinal disinsertion syndrome-lens subluxation, keratoconus
96. Retrolental fibroplasia (RLF)-cataracts, corneal opacification
97. Rieger syndrome (dysgenesis mesodermalis corneae et irides)-microcornea, corneal
opacities in Descemet membrane, dislocated lens
98. Romberg syndrome (facial hemiatrophy)-neuroparalytic keratitis, cataracts
99. Rothmund syndrome (telangiectasia-pigmentation-cataract syndrome)-cataract,
corneal lesions
*100. Rubella syndrome (German measles)-corneal haziness, cataracts, microcornea
101. Sabin-Feldman syndrome-posterior lenticonus, microcornea
102. Sanfilippo-Good syndrome (mucopolysaccharidosis III)-deposits in cornea and lens
103. Schafer syndrome (keratosis palmoplantaris syndrome)-lesions in the lower portion
of the cornea, cataract
104. Schaumann syndrome (sarcoidosis syndrome)-keratitis sicca, band-shaped keratitis,
complicated cataract
105. Scheie syndrome (mucopolysaccharidosis IS)-diffuse haze to marked corneal
clouding, cataracts
106. Siderosis (intraocular iron foreign body)-iron deposition in lens and cornea
107. Stannus cerebellar syndrome-corneal vascularization, corneal opacities, cataracts
108. Steroids-cataract, may worsen certain types of corneal infections
109. Stevens-Johnson syndrome (erythema multiforme exudativum)-keratitis, corneal
ulcers, cataracts, pannus
110. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)-keratopathy,
cataracts
111. Thioridazine-corneal and lens opacities
112. Toxic lens syndrome-pigment precipitation on the surface of an intraocular lens,
chronic uveitis
113. Trisomy syndrome-corneal and lens opacities
114. Turner syndrome (gonadal dysgenesis)-corneal nebulae, cataracts
115. Ultraviolet radiation-band keratopathy, keratitis, discoloring of lens
116. van Bogaert-Scherer-Epstein syndrome (familial hypercholesterolemia syndrome)-
lipid keratopathy, cataract, juvenile corneal arcus
117. von Recklinghausen syndrome (neurofibromatosis)-nodular swelling of corneal
nerves, cataracts
118. Waardenburg syndrome (interoculoiridodermatoauditive dysplasia)-microcornea,
cornea plana, lenticonus
119. Wagner syndrome (hereditary hyaloideoretinal degeneration and palatoschisis)-
corneal degeneration, including band-shaped keratopathy, cataracts
120. Ward syndrome (nevus jaw cyst syndrome)-congenital cataracts, congenital corneal
opacities
121. Wegener syndrome (Wegener granulomatosis)-corneal ulcer, corneal abscess,
cataract
122. Weil disease (leptospirosis)-keratitis, cataract
123. Werner syndrome (progeria of adults)-juvenile cataracts, bullous keratitis, trophic
corneal defects
*124. Wilson disease (hepatolenticular degeneration)-sunflower cataract, Kayser-
Fleischer ring
125. Yersiniosis-corneal ulcer, cataract
126. Zellweger syndrome (cerebrohepatorenal syndrome of Zellweger)-corneal opacities,
cataract
Dolan BJ, et al. Amiodarone keratopathy and lens opacities. J Am Optom Assoc 1985;
56:468-470.
Warburg M, et al. Autosomal recessive microcephaly, microcornea, congenital cataract,

mental retardation, optic atrophy, and hypogenitalism-Micro syndrome. Am J Dis Child
1993; 147:309-312.
DRUGS ASSOCIATED WITH CATARACTS
acetohexamide dexamethasone mepivacaine
Acetophenazine dextrothyroxine (?) meprednisone
(phenothiazine) diazoxide mescaline
acetylcholine dibucaine mesoridazine
adrenal cortex injection diethazine methdilazine
alcohol diethylpropion (?) methotrimeprazine
aldosterone diisopropyl methoxsalen (?)
allopurinol flurophosphate methylergonovine (?)
amiodarone (?) (DFP) methylprednisolone
amodiaquine doxorubicin methysergide (?)
azathioprine (?) droperidol (?) mitomycin C (topical)
Bacille Calmette-Guérin dyclonine (?)
(BCG) vaccine (?) echothiophate mitotane
beclomethasone ergocalciferol naltrexone
benoxinate ergonovine (?) naproxen (?)
benzodiazepine ergot (?) neostigmine
benzphetamine (?) ergotamine (?) nifedipine (?)
betamethasone ethopropazine oral contraceptives (?)
betaxolol (?) ethotoin oxygen
bleomycin etidocaine paramethasone
bupivacaine etretinate penicillamine (?)
busulfan fenfluramine (?) perazine
butaperazine fludrocortisone pericyazine
cactinomycin fluorometholone perphenazine
calcitriol (?) fluphenazine phenacaine
carbachol fluprednisolone phendimetrazine (?)
carbamazepine (?) ganciclovir phenmetrazine
carbromal(?) glyburide phentermine (?)
carphenazine gold phenytoin
chloroprocaine haloperidol (?) physostigmine
chloroquine hydrocortisone pilocarpine
chlorphentermine (?) hydroxychloroquine piperacetazine
chlorpromazine ibuprofen (?) piperazine (?)
chlorpromazine indomethacin (?) piperocaine
chlorpropamide isofluorphate prazosin (?)
chlorprothixene isotretinoin (?) prednisolone
cholecalciferol (?) levobunolol (?) prednisone (steroid)
clomiphene (?) lidocaine prilocaine
cobalt lithium carbonate(?) procaine
colchicine(?) lysergic acid prochlorperazine
cortisone diethylamide (LSD) promazine
danazol(?) lysergide promethazine
deferoxamine medrysone propiomazine
demecarium megestrol acetate propoxycaine
desoxycorticosterone mephenytoin (?) psilocybin
PUVA (psoralens and thiopropazate triflupromazine
ultraviolet A) (?) thioproperazine trimeprazine
quinacrine thioridazine trioxsalen (?)
silicone thiothixene troleandomycin (?)
sulfonamides (maternal timolol (?) urokinase (?)
ingestion) tolazamide verapamil (?)
sulindac (?) tolbutamide vitamin D2 (?)
tamoxifen triamcinolone vitamin D3 (?)
tetracaine trifluoperazine warfarin
thiethylperazine trifluperidol (?)
SYNDROMES AND DISEASES ASSOCIATED WITH CATARACTS
1. Aberfeld syndrome (blepharophimosis associated with generalized myopathy)

3. Addison syndrome (adrenal cortical insufficiency)
4. Albinism
6. Alopecia areata
*7. Alport syndrome (hereditary nephritis)
8. Alström disease (cataract and retinitis pigmentosa)
9. Andogsky syndrome (atopic cataract syndrome)
12. Apical malformations associated with cataracts
14. Arthrogryposis multiplex congenita
15. Aspergillosis
*16. Atopic dermatitis syndrome
17. Autosomal dominant foveal hypoplasia and presenile cataract syndrome (O'Donnell-
Pappas syndrome)
20. Bonnevie-Ullrich syndrome (pterygolymphangiectasia)
23. Caisson syndrome (bends)
25. Carotid artery syndrome
26. Cataract and hypertrophic neuropathy-autosomal recessive
27. Cataract with microcornea and coloboma of iris-autosomal dominant
28. Cataract, floriform-autosomal dominant
29. Cataract and cardiomyopathy-autosomal recessive
30. Cataract, congenital, or juvenile-autosomal recessive
31. Cataract, congenital total, with posterior sutural opacities in heterozygotes-X-linked
32. Cataract, congenital with absence deformity of leg-autosomal recessive
33. Cataract, congenital, with microcornea or slight microphthalmia-X-linked
34. Cataract, cortical, and congenital ichthyosis-autosomal recessive
35. Cataract, mental retardation, hypogonadism (Martsolf syndrome)
36. Cataract, microcephaly, arthrogryposis kyphosis syndrome (CAMAK syndrome)-
autosomal recessive
37. Cataract microcephaly, failure to thrive, kyphoscoliosis syndrome (CAMFAK
syndrome)-autosomal recessive
38. Cataract, nuclear and total nuclear-usually autosomal dominant rarely recessive
39. Cataract, zonular, and nystagmus-X-linked
40. Cat-eye syndrome (Schmid-Fraccaro syndrome)
41. Cerebral cholesterinosis (cerebrotendinous xanthomatosis)
42. Cerebellar ataxia, cataract, deafness, and dementia or psychosis
43. Cerebral palsy
45. Cerebrotendinous xanthomatosis
46. Cholera
47. Chromosome 13q partial deletion (long-arm) syndrome
48. Chromosomal 3; 18 translocation
49. Chromosome deletion (short-arm) syndrome
51. Cerebrooculofascioskeletal syndrome (COFS syndrome)
52. Congenital cataract and hypertrophic cardiomyopathy syndrome
53. Congenital cataract with oxycephaly (tower skull)
54. Congenital hemolytic icterus
55. Congenital ichthyosiform erythroderma
57. Conradi syndrome (stippled epiphyses syndrome)
58. Comedo-cataract
59. Craniofacial dysostosis (Crouzon disease)
61. Crome syndrome (congenital cataracts, epileptic fits, mental retardation, small
stature)
62. Cushing syndrome
63. Cytomegalovirus
64. Darier-White syndrome (keratosis follicularis)
65. DeBarsy syndrome
66. Dermatitis herpetiformis
68. Diarrhea
69. Diphtheria
70. Ectodermal dysplasia
71. Edward syndrome
76. Fabry disease (diffuse angiokeratosis)
77. Familial congenital cataracts, microcornea, abnormal irides, nystagmus, and
glaucoma syndrome
78. Familial congenital cataract, nonprogressive neurologic disorders, and mental
deficiency syndrome
79. Familial histiocytic dermatoarthritis syndrome
80. Familial hypogonadism syndrome
81. Familial t(2;16) translocation
83. Folling syndrome (phenylketonuria)
84. François dyscephalic syndrome (Hallermann-Streiff syndrome)
86. Fuchs syndrome (I) (heterochromic cyclitis syndrome)
87. Galactokinase deficiency-autosomal recessive
*88. Galactosemia-transferase deficiency
90. Goldscheider syndrome (epidermolysis bullosa)
91. Gorlin-Goltz syndrome (multiple basal cell nevi syndrome)
94. Hagberg-Santavuori syndrome (neuronal ceroid-lipofuscinosis)
95. Hallermann-Streiff syndrome (oculomandibulofacial dyscephaly)
96. Hand-Schüller-Christian syndrome (histiocytosis X)
97. Harada disease (uveitis-vitiligo-alopecia-poliosis syndrome)
98. Heerfordt syndrome (uveoparotid fever)
99. Hemifacial microsomia syndrome (François- Haustrate syndrome)
100. Herpes simplex virus
101. Hilding syndrome (destructive iridocyclitis and multiple joint dislocations)
102. Hodgkin disease
103. Homocystinuria
104. Hookworm disease
105. Hruby-Irvine-Gass syndrome (cystoid maculopathy following cataract extraction
with vitreous loss)
106. Hutchinson-Gilford syndrome (progeria)
107. Hydatid cyst
108. Hypercalcemia (adult)
109. Hypercalcemia (infantile) with mental retardation (supravalvular aortic stenosis
syndrome)
110. Hyperprolactinemia
111. Hypertrophic cardiomyopathy
114. Hypocalcemia
115. Hypoglycemia
118. Incontinentia pigmenti achromians
119. Infantile hypoglycemia (male)
120. Influenza
121. Infrared radiation
122. Intrauterine infections
A. herpes virus
B. mumps
C. rubella
D. toxoplasmosis
E. vaccinia
124. Jadassohn-Lewandowsky syndrome (pachyonychia congenita)
125. Karsch-Neugebauer syndrome (nystagmus-split hand syndrome)
126. Klippel-Trenaunay-Weber syndrome (angioosteohypertrophy syndrome)
128. Kussmaul disease (periarteritis nodosa)
129. Kyrle disease (hyperkeratosis penetrans)
130. Lanzieri syndrome (craniofacial malformations)
131. Laser treatment for retinopathy of prematurity
132. Laurence-Moon-Biedl syndrome (retinitis pigmentosa-polydactyly-adiposogenital)
133. Leber syndrome (optic atrophy-amaurosis-pituitary syndrome)
134. Leiomyoma
136. Lightning
137. Listerellosis
*138. Lowe syndrome (oculocerebrorenal syndrome)
139. Majewski syndrome (short-rib polydactyly syndrome)
140. Malaria
141. Male Turner syndrome (Noonan syndrome)
142. Malignant hyperpyrexia syndrome
144. Mannosidosis
145. Maple-syrup urine disease (branched-chain ketoaciduria
146. Marfan syndrome (arachnodactyly dystrophia mesodermaliscongenita)
147. Marinesco-Sjögren syndrome (congenital cataract-oligophrenia syndrome)
148. Marshall syndrome (atypical ectodermal dysplasia)
149. Martsolf syndrome
150. Matsoukas syndrome (oculocerebroarticuloskeletal syndrome)
152. Microcephaly, microphthalmia, cataracts, and joint contractures syndrome
153. Microphthalmia-congenital anterior polar cataract syndrome-autosomal dominant
154. Micro syndrome
155. Miller syndrome (Wilms aniridia syndrome)
156. Monilethrix
157. Morgan syndrome (intracranial exostosis)
158. Morquio-Brailsford syndrome (mucopolysaccharidoses IV)
160. Myopic (high)
162. Nail-patella syndrome (Little syndrome)
163. Nance-Horan syndrome (cataract-dental syndrome)
164. Neurodermatitis
165. Neurofibromatosis 1 (von Recklinghausen syndrome)
167. Nieden syndrome (telangiectasia-cataract syndrome)
168. Norrie disease
170. Optic atrophy, cataract, and neurologic disorder-autosomal dominant
171. Osteogenesis imperfecta congenita, microcephaly, and cataracts-autosomal recessive
173. Oxycephaly
174. Pachyonychia congenita syndrome
175. Paget syndrome (idiopathic hyperphosphatasemia)
177. Partial trisomy 10q trisomy
178. Passow syndrome (syringomyelia)
179. Patau syndrome
180. Pellagra (avitaminosis B2)
182. Pernicious anemia syndrome (vitamin B12 deficiency)
184. Prader-Labhart-Willi syndrome (hypogenital dystrophy with diabetic tendency)
185. Pseudoexfoliation syndrome
187. Radiation
188. Reese-Ellsworth syndrome (anterior chamber cleavage syndrome)
190. Renal failure (chronic)
191. Renal transplantation
192. Retinal ischemic infarction syndrome
*193. Retinitis pigmentosa-deafness-ataxia syndrome
194. Rhizomelic chondrodysplasia punctata
195. Riboflavin deficiency syndrome (oculoorogenital syndrome)
196. Ring chromosome in the D group
197. Robert syndrome
198. Robert pseudothalidomide syndrome
200. Rothmund syndrome (infantile poikiloderma)
*201. Roy syndrome I (unilateral cataract associated with smoking)
202. Roy syndrome II-nuclear cataract associated with smoking
203. Rubeola (measles)
204. Rubinstein-Taybi syndrome (broad-thumbs syndrome)
205. Scaphocephaly syndrome (craniofacial dysostoses)
206. Schaefer syndrome (congenital dyskeratosis)
207. Schwartz syndrome
208. Scurvy (avitaminosis C)
209. Sickle cell disease (Herrick syndrome)
210. Siemen syndrome (congenital atrophy of the skin)
212. Sjögren-Larsson syndrome (oligophrenia ichthyosis)
213. Smith-Lemli-Opitz syndrome
214. Split-hand with congenital nystagmus, fundal changes, cataracts-autosomal
dominant
215. Spondyloepiphyseal dysplasia (SED) dwarfism
216. Stannus cerebellar syndrome (vitamin B2 deficiency)
217. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)
*218. Still disease (juvenile rheumatoid arthritis)
219. Thrombocytopenia-absent radius (TAR) syndrome
220. Toxocariasis (nematode ophthalmia syndrome)
221. Treacher Collins syndrome (mandibulofacial dysostosis)
222. Trichomegaly, spherocytosis, and cataract -autosomal dominant
223. Trichorrhexis nodosa (argininosuccinicaciduria)
224. Trisomy 13 syndrome (Patau syndrome)
225. Trisomy 16 syndrome (Edward syndrome)
227. Trisomy 21 (Down syndrome)
228. Tuomaala-Haapanen syndrome
230. Tyrosinosis (Hanhart syndrome)
231. Usher syndrome (hereditary retinitis pigmentosa-deafness syndrome)
232. Uvea-touch syndrome
233. Van der Hoeve syndrome (brittle-bone disease)
234. Van Bogaert-Scherer-Epstein syndrome (primary hyperlipidemia)
235. Varicella infection
283. Warburg syndrome (hydrocephalus, agyria, and absent cortical laminar retinal
dysplasia with or without encephalocele)
239. Ward syndrome (nevus-jaw cyst syndrome)
241. Weil disease (leptospirosis)
242. Werner syndrome (scleropoililoderma)
244. Yersiniosis
246. 31 syndrome
247. 4p syndrome
248. l8p syndrome
249. l8q syndrome
Christiansen JP. Bradford JD. Cataract in infants treatment with argon laser
photocoagulation for threshold retinopathy of prematurity. Am J Ophthalmol 1995;
119:175-180.
Francis PJ. et al. Visual outcome in patients with isolated autosomal dominant congenital
cataract. Ophthalmology 2001; 108:1104-1108.
Ng JS. et al. Ocular complications of pediatric bone marrow transplantation.

LENTICULOCORNEAL ADHERENCE (LENS ADJACENT TO

ENDOTHELIUM
OF CORNEA)
1. Acquired anterior corneal disease as ulcer with perforation or trauma

2. Aniridia
3. Peters anomaly (oculodental syndrome)
4. Rieger anomaly (dysgenesis mesostromalis)
Kivlin JD, et al. Peters' anomaly as a consequence of genetic and nongenetic syndromes.
Waring G, et al. Ultrastructure and successful keratoplasty of sclerocornea in Mieten's

SPASM OF ACCOMMODATION
This condition involves increased tone of ciliary body with increased convexity of
crystalline lens (see p. 619-622).
1. Alcoholism
2. Cerebrovascular accident
3. Contusion injury to the globe or head
4. Cyclic oculomotor palsy or spasm
6. Drugs, such as aceclidine, acetylcholine, carbachol, demecarium, DFP, digitalis,
echothiophate, guanethidine, isoflurophate, methylene blue, morphine, neostigmine,
opium, physostigmine, pilocarpine
7. Fatigue cramp of overworked ciliary muscle; most frequent with compound hyperopia
and mixed astigmatism associated with anisometropia
8. Graves disease (hyperthyroidism, Basedow syndrome)
9. Infectious, such as diphtheria, helminthic infestations, or sinus disease
10. Irritative lesions of brain stem and oculomotor trunk, such as epidemic encephalitis,
tabes, meningitis, influenza, scleritis, measles, or orbital inflammation
11. Middle cerebral artery occlusion
12. Ocular inflammation, such as ciliary muscle irritant
13. Pineal tumor
14. Reflex irritation, such as in trigeminal neuralgia
15. Sympathetic paralysis
16. Trauma
Ohtsuka K, et al. Accommodation and convergence insufficiency with left middle

cerebral artery occlusion. Am J Ophthalmol 1988; 106:60-64.
Wilkins, 1988.
PARESIS OF ACCOMMODATION
This condition involves partial or total loss of physiologic ability to change the
shape of the lens and thus the focus of the eye (see Mydriasis, p. 622-623); this ability is
related to age (see Acquired Hyperopia, p. 344-348).
*1. Presbyopia-gradual decrease in amplitude of accommodation related to age

2. Accommodative insufficiency
A. Asthenic individuals
B. Illness or debilitation, including intestinal toxemia, tuberculosis, influenza,
whooping cough, measles, and tonsillar and dental infections
C. Anemia
D. Overwork
E. Whiplash injury
3. Ciliary body aplasia-with or without pupillary and iris abnormalities
4. Iridocyclitis-acute and chronic
5. Glaucoma with atrophy of ciliary body
6. Choroidal metastasis with suprachoroidal extension
7. Trauma, such as tears in iris sphincter, tears at root of iris, or recession of the anterior
chamber angle with posterior displacement of the ciliary attachment and ocular
hypotension
8. Rupture of zonular fibers and partial subluxation of lens
carbinoxamine hexamethonium
carbon dioxide hexocyclium
carphenazine homatropine
chloramphenicol hydrochlorothiazide
chlordiazepoxide hydrocortisone
chlorisondamine hydroflumethiazide
chlorothiazide hydromorphone
chlorphenoxamine hydroxyamphetamine
chlorpromazine imipramine
acetazolamide chlorthalidone indapamide
acetophenazine cimetidine iodide and iodine
adiphenine clemastine solutions and
alcohol clidinium compounds
alprazolam clomipramine isoniazid
ambutonium clonazepam isopropamide
aminosalicylate (?) clorazepate maprotiline
aminosalicylic acid (?) cocaine marijuana
amitriptyline cortisone mecamylamine
amodiaquine cyclopentolate medrysone
amoxapine cyclothiazide mepenzolate
amphetamine cycrimine meprobamate
anisindione desipramine mescaline
anisotropine dexamethasone mesoridazine
antazoline dextroamphetamine methacholine
atropine diacetylmorphine methamphetamine
belladonna diazepam methantheline
bendroflumethiazide dibucaine methaqualone
benzathine penicillin G dichlorphenamide methazolamide
benzphetamine dicyclomine methdilazine
benzthiazide diethazine methixene
benztropine diphemanil methotrimeprazine
betamethasone diphenadione methscopolamine
bethanechol diphenhydramine methyclothiazide
biperiden diphenylpyraline methylatropine nitrate
bromide emetine methylene blue
butaperazine ergot methyprylon
captopril (?) ethopropazine methysergide
caramiphen fluorometholone metolazone
carbachol fluphenazine mianserin
carbamazepine glycopyrrolate midazolam
morphine piroxicam (?) temazepam
nalidixic acid poldine tetanus immune globulin
naproxen polythiazide tetanus toxoid
nitrazepam potassium penicillin G tetracaine
nortriptyline potassium penicillin V tetraethylammonium
opium potassium phenethicillin tetrahydrocannabinol
orphenadrine pralidoxime thiethylperazine
oxazepam prazepam thiopropazate
oxymorphone prednisolone thioproperazine
oxyphencyclimine primidone thioridazine
oxyphenonium procaine penicillin G thiothixene
pargyline procarbazine triazolam
pentazocine prochlorperazine trichlormethiazide
pentolinium procyclidine trichloroethylene
perazine promazine tridihexethyl
periciazine promethazine trifluoperazine
perphenazine propantheline trifluperidol
phendimetrazine propiomazine triflupromazine
phenindione propranolol trihexyphenidyl
phenmetrazine protriptyline trimeprazine
phentermine psilocybin trimethaphan
pilocarpine pyrilamine trimethidinium
pimozide quinethazone trimipramine
pipenzolate radioactive iodides tripelennamine
piperacetazine rubella virus vaccine tropicamide
piperazine (live) vinblastine
piperidolate scopolamine vincristine
piperocaine streptomycin
11. Neurogenic causes

A. Infectious conditions
(1) Epidemic encephalitis
(2) Anterior poliomyelitis
(3) Exanthemas and acute infections, such as scarlet fever, mumps,
measles, influenza, typhoid fever, dengue fever, viral hepatitis, amebic
dysentery, and malaria
(4) Herpes zoster
(5) Syphilis (lues)
(6) Tuberculosis
(8) Focal infections, such as from teeth or nasal sinuses
B. Toxic conditions
(1) Alcohol
(2) Lead
(3) Arsenic
(4) Carbon monoxide
(5) Diphtheritic paralysis
(6) Botulism
(7) Extensive bum
(8) Snake venom
C. Degenerative conditions
(1) Congenital hereditary ophthalmoplegia
(2) Progressive congenital ophthalmoplegia
(3) Hereditary ataxia
(4) Myotonic dystrophy (Curschmann-Steinert syndrome)
(5) Myasthenia gravis
D. Metabolic conditions
(1) Acute hemorrhagic anterior polioencephalitis of Wernicke
(2) Diabetes mellitus
(3) Lactation
(4) Following pregnancy
E. Isolated internal ophthalmoplegia
F. Isolated failure of near reflex, such as with inverse Argyll-Robertson pupil
G. Lesions of parasympathetic nuclei in midbrain
(1) Encephalitis
(2) Pineal tumor
(3) Other signs of mesencephalic disease, including multiple sclerosis,
infectious polyneuropathy, and vascular lesions
(4) Syphilis-bilateral
H. Trauma to head or neck
(1) Cerebral concussion
(2) Craniocervical extension injuries
Pau FH. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical,
1988.
Wilkins, 1985.
14
Vitreous
CONTENTS
Pseudodetachment of vitreous 421
Anterior vitreous detachment 421
Posterior vitreous detachment 422
Vitreous hemorrhage 424
Vitreous opacities 429
Persistent hyperplastic primary vitreous 430
Beads in vitreous 430
Asteroid hyalosis versus synchysis scintillans 431
Complications following operative vitreous loss 431
Postoperative vitreous retraction 431
Vitreous cyst 432
Vitreous liquefaction 432
PSEUDODETACHMENT OF VITREOUS (CONDITIONS SIMULATING

DETACHMENT OF VITREOUS)
1. Enormous cavity in the vitreous body with a relatively thin posterior wall
2. Membranous formations within the vitreous associated with uveitis and hemorrhage
3. Outline of the ascending portion of Cloquet canal just anterior to the disc
Tolentino FI. et al. Vitreoretinal disorders: diagnoses and management. Philadelphia:

WB Saunders, 1976.
ANTERIOR VITREOUS DETACHMENT

In this condition, the anterior vitreous cortex may be separated from the posterior
lens or posterior zonular fibers.
1. Retrolenticular-usually caused by vitreous shrinkage

*A. Trauma (most common)
B. Hemorrhage-usually secondary to trauma
C. Senescence (rare)
D. Inflammation
E. Retinal detachment (see p. 487)
*F. Iatrogenic after injection of vitreous substitutes (gas)
2. Retroocular
A. Vitreous shrinkage (see p. 432)
B. Ciliary body tumor
C. Blood
D. Exudate
3. Retrolenticular and retroocular combined occurs with rupture of the hyaloideocapsular
ligament
Tolentino FI, et al. Vitreoretinal disorders: diagnosis and management. Philadelphia:
WB Saunders, 1976.
POSTERIOR VITREOUS DETACHMENT
1. Complete posterior detachment

A. Simple detachment-occurs in young persons
(1) Exudate from chorioretinal focus
(2) Hemorrhage between the vitreous and the retina
(3) Retraction of the cortical vitreous caused by exudate within the
vitreous
(4) Vitreous hemorrhage in a young individual with vitreous shrinking due
to thrombosis of central retinal vein, retinal neovascularization
B. Complete posterior detachment with collapse
(1) Senescent changes are primary cause
(2) Uveitis
(3) Trauma
(4) Hemorrhage
(5) Sodium hyaluronate
C. Funnel-shaped posterior detachment
(1) Perforating injuries of globe
(2) Retinal neovascularization
(3) Massive vitreous detachment
D. Atypical complete posterior detachment-residual adherence of vitreous to a
peripheral retinal area
(1) Focus of chorioretinitis
(2) Following cataract extraction with loss of vitreous
(3) Following perforating wounds
(4) Posterior uveitis with inflammatory cells
2. Partial posterior detachment (unusual)
A. Superior detachment-primarily a senescent change; generally forerunner of
posterior vitreous detachment with collapse
B. Partial posterior detachment (not infrequent)
(1) Preretinal hemorrhage
(2) Retinal neovascularization
C. Partial lateral or partial inferior detachment
(1) Focus of choroiditis
(2) Circumscribed retinal periphlebitis
(3) Intraocular foreign body
Foos RY, et al. Posterior vitreous detachment in diabetic subjects. Ophthalmology 1980;
87: 122.
Jaffe NS. The vitreous in clinical ophthalmology. St. Louis: CV Mosby, 1969.
Nirankari VS, et al. Pseudo-vitreous hemorrhage: a new intraoperative complication of
sodium hyaluronate. Ophthalmic Surg Lasers 1981; 12:503.
Extracted Table Posterior Vitreous Detachment
VITREOUS HEMORRHAGE
1. Acquired lues (syphilis)

2. Arsenic toxicity
3. Ascariasis
4. Avulsed retinal vessel syndrome
5. Battered baby syndrome (Silverman syndrome)
7. Blood disease-retinal hemorrhage breaking into vitreous
A. Anemias
(1) Aplastic anemia
(2) Hemolytic anemia
(3) Hypochromic anemia
(4) Pernicious anemia
B. Dysproteinemias - macroglobulins and cryoglobulins
C. Hemophilia associated with trauma
D. Leukemias
E. Multiple myeloma (Kahler disease)
F. Polycythemia vera (Vaquez disease)
G. Thrombocytopenic purpura
8. Coats disease (retinal telangiectasia)
9. Collagen disease
A. Dermatomyositis
B. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
C. Polyarteritis nodosa (Kussmaul disease)
D. Scleroderma (progressive systemic sclerosis)
10. Complete posterior vitreous detachment with collapse (10%-15% at time of event)
11. Cysticercosis
12. Dengue fever
13. Diabetes mellitus-proliferative retinopathy
14. Dislocation of intraocular lenses
15. Disseminated intravascular coagulation
16. Drusen of optic disc
17. Eales disease
18. Exudative age-related macular degeneration
19. Familial exudative vitreoretinopathy
20. Ganciclovir Implant
21. Gronblad-Strandberg syndrome (systemic elastodystrophy)
22. Hemorrhages in the newborn
A. Hemorrhagic disease of the newborn factor VII and prothrombin deficiency
B. Persistent vessels of the hyaloid system
C. Retinal hemorrhage of newborn breaking through to vitreous cavity
23. Iatrogenic globe perforation associated with strabismus surgery
24. Indomethacin reaction
25. Influenza
26. Intraocular foreign body
28. Hypertension (venous occlusive disease)
29. Juvenile retinoschisis
*30. Macroaneurysm (retinal arterial)
31. Malaria
33. Migration from anterior bleeding as from angle-supported, iris-supported, or posterior
chamber lenses
*34. Neovascularization following vascular occlusion (primarily venous occlusive
disease)
35. Neovascularization of cataract wound
36. Ocular ischemic syndrome with neovascularization of disc
37. Pars planitis
38. Persistent hyaloid artery
39. Persistent hyperplastic primary vitreous (PHPV)
*40. Posterior vitreous detachment (PVD)
41. Purtscher disease (traumatic retinal angiopathy)
42. Retinal angiomatosis (von Hippel disease)
*43. Retinal break or tear with or without retinal detachment and avulsed retinal vessels
*44. Retinal hemorrhage, including vein occlusion and sickle retinopathy, arterial
macroaneurysm
45. Retinal tacks (intrusion)
46. Retinoblastoma
47. Retinopathy of prematurity-proliferative stage
48. Scleral buckle (intrusion)
49. Sleep apnea
50. Sickle cell disease (Herrick syndrome)-SA, SS, or SC
51. Surgical cataract complication with lenticular fragments dislocated into vitreous
52. Terson syndrome of associated vitreous and subarachnoid hemorrhage syndrome
53. Thalassemia (Cooley anemia)
54. Thromboangiitis obliterans (Buerger disease)
55. Tissue plasminogen activator (t-PA)
56. Tuberous sclerosis
57. von Hippel-Lindau disease (angiomatosis retinae)
58. von Willebrand syndrome
59. Trauma
60. Traumatic asphyxia
61. Tuberculosis
62. Uveitis (associated with)
63. Varicella zoster
Kokame GT. Vitreous hemorrhage after intravitreal tissue plasminogen activator (t-PA)
and pneumatic displacement of submacular hemorrhage. Am J Ophthalmol 2000;
129:546-547.
Kuhn F, et al. Terson syndrome. Ophthalmology 1998; 105:472-477.
Potter MJ, et al. Vitrectomy for pars planitis complicated by vitreous hemorrhage: visual
outcome and long-term follow-up. Am J Ophthalmol 2001; 131:514-515.
Spraul CW, Grossniklaus HE. Vitreous hemorrhage. Surv Opthalmol 1997; 42:1, 3-6.
Extracted Table Vitreous hemorrhage
VITREOUS OPACITIES
1. Opaque sheets anterior to the vitreous

A. Elschnig pearls after extracapsular cataract extraction or needling (posterior
capsule opacification)
B. Normal posterior capsule-often following extracapsular cataract extraction or
needling
C. Soemmerring ring following extracapsular cataract extraction or needling
D. Vitreous adhesions to iris, capsule, or intraocular lens (IOL) after cataract
extraction with vitreous loss
2. Pseudoglioma-leukokoria (see p. 357)
3. Scattered opacities
A. Amyloid disease-rare (seen in older persons)
B. Ankylosing spondylitis
C. Coagula of the colloid basis of the gel
D. Crystalline deposits
(1) Asteroid hyalosis
(2) Synchysis scintillans
E. Endophthalmitis
F. Heterochromic uveitis-in persons 20 to 50 years of age; of all uveitis, iris
atrophy, lens changes
G. Myeloma, multiple-rare: in persons 50 to 70 years old, associated with bone
pain, anemia
H. Myopia, severe
I. Pigment cells-posttraumatic (hemorrhage), senile, or melanotic, associated with
rhegmatogenous retinal detachment.
J. Protein coagulaplasmoid vitreous
(1) Choroidal tumors (very rare-reported in metastatic breast cancer once)
(2) Contusions
(3) Intermediate uveitis (pars planitis)
(4) Retinochoroiditis
K. Red blood cells (see vitreous hemorrhage, p. 424)
L. Snowball opacities-rare, associated with pars planitis or sarcoidosis,
endophthalmitis (indolent)
M. Tissue cells-epithelial, histiocytic, glial
N. Toxoplasmosis-active
O. Tumor cells-retinoblastoma in older child, reticulum cell sarcoma (older
persons)
P. Vitreous degeneration-Wagner disease, Ehlers-Danlos syndrome, and Marfan
syndrome, senescent aging changes, myopia
Q. Whipple disease
R. White blood cells-inflammatory disease, vitreitis
S. Retinitis pigmentosa
4. Single opacities
A. Anterior hyaloid remnant (Mittendorf dot)-25% normal eyes, dot on posterior
lens surface
B. Hyaloid remnants (uncommon)-persistent hyperplastic primary vitreous
C. Foreign body-history of trauma or surgery
D. Dislocated lens (see p. 401)
E. Parasitic cysts
(1) Hydatid disease (echinococcosis)-rare, children and young adults,
tropical
(2) Cysticercosis-rare
F. Vitreous detachment-common in older or myopic persons
Durant WJ, et al. Vitrectomy and Whipple's disease. Arch Ophthalmol 1984; 102:851.
Hong PH, et al. Vitrectomy for large vitreous opacity in retinitis pigmentosa. Am J
Ophthalmol 2001; 131:133-134.
Recchia AE, et al. Endophthalmitis after pediatric strabismus surgery. Arch Ophthalmol
2000; 118:939-944.
Sandgren O, et al. Vitreous amyloidosis associated with homozygosity for the

transthyretin methionine gene. Arch Ophthalmol 1990; 108:1586.
PERSISTENT HYPERPLASTIC PRIMARY VITREOUS
1. Cerebrooculodysplasia-muscular dystrophy
3. Incontinentia pigmenti
4. Norrie disease
5. Septooptic pituitary
6. Sporadic unilateral and isolated finding
7. Trisomy 13
8. Warburg syndrome
Katsuya-Lauer A, et al. Persistent hyperplastic primary vitreous associated with septo-
optic-pituitary dysplasia and schizencephaly. Arch Ophthalmol 2000; 118:578-580.
BEADS IN VITREOUS (SNOWBALLS IN VITREOUS)
1. African eye-worm disease (loiasis)

4. Birdshot retinochoroidopathy
6. Familial exudative vitreoretinopathy (Criswick-Schepens syndrome)
7. Haemophilus influenzae
8. Irvine syndrome
10. Ocular toxocariasis
11. Ocular toxoplasmosis
13. Pars planitis
14. Retinoblastoma
15. Sarcoidosis
16. Severe uveitis
17. Sympathetic ophthalmia
18. Toxic lens syndrome
19. Typhus (Japanese river fever)
Noda S, et al. Patients with asteroid hyalosis and visible floaters. Jpn J Ophthalmol 1995;
37:452-455.
Schlaegel TF. The uvea. Arch Ophthalmol 1971; 85:635.
COMPLICATIONS FOLLOWING OPERATIVE VITREOUS LOSS
1. Inflammatory complications
A. Irritable eye (chronically)
B. Recurrent or persistent uveitis
C. Vitreitis with vitreous opacities
2. Wound complications
A. Epithelial invasion or downgrowth
B. Fibrous ingrowth
C. Fistula or gaping of wound (with or without vitreous wick syndrome)
*D. Infection or endophthalrnitis
E. Excessive astigmatism
3. Corneal complications
A. Corneal edema (vitreocorneal touch)
B. Bullous keratopathy
C. Corneal opacification
4. Secondary glaucoma
A. Vitreous obstruction of anterior chamber angle
B. Pupillary block (iridohyaloid adhesions, anterior hyaloid displacement, uveitis)
C. Iris and vitreous adherence to wound (peripheral anterior synechiae)
5. Fibroblastic and traction phenomena
A. Pupillary membrane
B. Pupillary distortion- "peaked" or updrawn synechiae
C. Cystoid macular edema (CME)
E. Optic neuritis or papilledema
F. Vitreous hemorrhage
G. Posterior vitreous detachment
Krupin T, Kolker AG. Atlas of complications in ophthalmic surgery. St. Louis: CV

Mosby, 1993.
Peyman GA, Shulman JA. Intravitreal surgery: principles and practice. New York:
Appleton-Lange, 1994.
POSTOPERATIVE VITREOUS RETRACTION
Usually, this condition is manifested by circular equatorial retinal fold or star-

shaped retinal fold.
1. Accidental perforation of the sclera at operation, which may be associated with

hemorrhage and loss of vitreous resulting in a pathologic formation of new epiretinal
membrane or proliferative vitreoretinopathy
2. Giant retinal breaks allowing a large area of direct contact between the choroid and the
vitreous
3. Perforating diathermy and excessively strong or repeated applications of superficial
diathermy, which may cause vitreous hemorrhage or thermal injury to the vitreous;
impairment of chorioretinal blood circulation may result in exudation and hemorrhage
into the vitreous
4. Venous stasis caused by the compression of vortex veins by the indentation resulting
from a buckling procedure
Jaffe NS. The vitreous in clinical ophthalmology. St. Louis: CV Mosby, 1969.
VITREOUS CYST (CYSTIC STRUCTURE IN VITREOUS BODY)
1. Congenital (developmental)-may be associated with hyaloid remnants

2. Acquired
A. Infectious cyst
(1) Coenurosis (Coenurus cerebralis larva of dog tapeworm)
(2) Luetic retinochoroiditis
(3) Toxoplasmosis
B. Myopia
C. Parasitic cysts
(1) Cysticercosis-rare
(2) Echinococcosis
(3) Hydatid disease (echinococcosis)-rare, children and young
adults, tropical
(4) Nematode cyst (toxocariasis)
D. Pigmentary retinopathy
E. Retinal detachment
F. Trauma
Cardillo JA, et al. Post-traumatic proliferative vitreoretinopathy. Ophthalmology 1997;

104:1166-1173.
Flynn WJ, Carlson DW. Pigmented vitreous cyst. Arch Ophthal 1994; 112:1113.
Nussenblat RB, Palestine AG. Uveitis fundamentals and clinical practice. Chicago:
Yearbook Medical, 1989.
VITREOUS LIQUEFACTION
1. Myopia
2. Peripheral uveitis
4. Spontaneous
5. Trauma
6. With aging
7. With vitreous traction such as Wagner disease
Nussenblatt RB, Palestin AG. Uveitis: fundamental and clinical practice. Chicago:
Yearbook Medical, 1989.
Takhashi M, et al. Biomicroscopic evaluation and photography of liquefied vitreous in

some vitreoretinal disorders. Arch Ophthalmol 1981; 99:1555.
15
Retina
CONTENTS
Anatomic classification of macular diseases 435

Bilateral macular lesions 439
Pseudomacular edema 439
Macular edema 439
Absence of foveal reflex 442
Macular pucker 442
Macular exudates and hemorrhages 443
Macular star or stellate retinopathy 443
Retinociliary vein 446
Cherry-red spot in macula 446
Macular hemorrhage 447
Parafoveal telangiectasia 449
Microhemorrhagic maculopathy 449
Macular cyst 449
Macular hole 449
Macular coloboma 450
Elevated macular lesion 451
Heterotopia of the macula 451
White or yellow flat macular lesion and pigmentary change 451
Pigmentary changes in macula 452
Bull's-eye macular lesion 454
Macular wisps and foveolar splinter 454
Macular hypoplasia 454
Premacular subhyaloid hemorrhage 455
Retinal vascular tortuosity 455
Venous beading 456
Ophthalmodynamometry 456
Pulsation of retinal arteriole 457
Retinal artery occlusion 457
Localized arterial narrowing 462
Generalized arterial narrowing 462
Periarteritis retinalis segmentalis 464
Frosted branch angiitis 464
Sheathing of retinal veins 464
Absent venous pulsations 465
Dilated retinal veins 466
Tortuosity of retinal veins and hypoplasia of optic nerves 468
Central retinal vein occlusion 468
Dilated retinal veins and retinal hemorrhages 473
Retinal hemorrhages 474
Large hemorrhages in fundus of an infant or young child 479
Retinovitreal hemorrhage in a young adult 479
Retinal hemorrhage with central white spot (Roth spot) 479
Microaneurysms of retina 483
Retinal arteriovenous shunt at the arteriovenous crossing 486
Macroaneurysms of retinal arteries 486
Retinal neovascularization 486
Predisposition to rhegmatogenous retinal detachment 486
Retinal detachment 487
Syndromes and diseases associated with retinal detachment 487
Retinal folds 491
Cotton-wool spots 491
Hard exudates 495
Retinal exudate and hemorrhage 497
Retinitis or pseudoretinitis pigmentosa 497
Lesions confused with retinoblastoma 502
Diagnostic table-Single dark fundus lesion 503
Single white lesion of retina 505
Pale fundus lesions 505
Medullated nerve fibers 507
Pigmented fundus lesions 507
Cholesterol emboli of retina 509
Retinal microemboli 510
Lipemia retinalis 510
Hemorrhagic or serous exudates beneath pigment epithelium 511
Retinal vascular tumors and angiomatosis retinae syndromes 511
Traumatic retinopathies 513
Retinal "sea-fans" 514
Retinal vessels displaced temporally 514
Retinal vessels displaced nasally 514
Peripheral fundus lesions 515
Retinal disease associated with corneal problems 517
Retinal lesions associated with deafness 521
Subretinal fibrosis 521
Epiretinal membranes 522
Linear streaks pattern in fundus 522
Yellow-orange lesions of subretinal fundus 522
Talc retinopathy 523
Crystalline retinopathy 523
Pulfrich stereo-illusion phenomenon 523
Parafoveal telangiectasia 523
Hereditary pediatric retinal degenerations 524
Reticular pattern of dark lines in fundus 524
Retinal pigment epithelial tears 524
Retinal pigment epithelial folds 525
Mizuo phenomenon 525
White-dot fovea 525
ANATOMIC CLASSIFICATION OF MACULAR DISEASES
1. Vitreoretinal surface
A. Preretinal hemorrhage and subinternal limiting membrane hemorrhage
B. Vitreous traction on the macula
C. Epiretinal membrane and macular pucker
2. Nerve fiber-ganglion cell layers
A. Hereditary cerebromacular degeneration
(1) Sphingolipidoses
a. Tay-Sachs disease (GM2-gangliosidosis type I)
b. Sandhoff disease (GM MDSD2-gangliosidosis type II)
c. Niemann-Pick disease type A (essential lipoid histiocytosis)
d. Niemann-Pick disease type B (sea-blue histiocyte syndrome)
e. Lactoside ceramidosis
f. Metachromatic leukodystrophy (arylsulfatase A deficiency)
g. Gaucher disease (glucocerebroside storage disease)
h. Farber lipogranulomatosis
i. Generalized gangliosidosis (GM1-gangliosidosis type I)
j. Mucolipidosis I (lipomucopolysaccharidosis)
(2) Goldberg disease (unclassified syndrome with features of
mucopolysaccharidoses, sphingolipidoses, and mucolipidoses)
(3) Ceroid lipofuscinosis
a. Hagberg-Santevuori (infantile)
b. Jansky-Bielschowsky disease (late infantile)
c. Spielmeyer-Vogt Batten
B. Vitreoretinal dystrophies
(1) Macular degeneration in congenital hereditary x-linked retinoschisis
(2) Goldmann-Favre syndrome (vitreotapetoretinal degeneration)-
recessive
3. Nerve fiber, ganglion cell, inner plexiform, inner nuclear, outer plexiform layers
A. Ischemia secondary to inadequate perfusion of retinal vessels
(1) Branch artery occlusion
(2) Branch vein occlusion
4. Outer plexiform layer
A. Cystoid macular degeneration (see p. 439)
(1) With retinal vascular leakage
a. Acute nongranulomatous iridocyclitis
b. Acute cyclitis
c. Hypertension
d. Medication (epinephrine, nicotinic acid)
e. Neoproliferative diabetic retinopathy
f. Pars planitis
g. Postoperative (Irvine-Gass syndrome)
h. Radiation retinopathy
i. Retinitis pigmentosa
j. Sarcoidosis
k. Vascular anomalies
(2) Without obvious retinal vascular leakage
a. Vitreous traction on the macula
b. Serous detachment of sensory epithelium
c. Serous detachment of pigment epithelium
d. Hemorrhagic detachment of macula
e. Choroidal tumors
B. Lipid deposits in macula secondary to vascular disease in retina
(1) Stellate retinopathy (see p. 443)
a. Hypertensive retinopathy
b. Diabetic retinopathy
c. Coats disease (retinal telangiectasia)
d. Trauma-ocular or cerebral
e. Retinal artery or vein occlusion (see p. 457 and 468)
f. Retinal periphlebitis
g. Juxtapapillary choroiditis
h. Papilledema (see p. 593-601)
i. Angiomatosis retinae
j. Papillitis (see p. 578-585, 587-588)
k. Acute, febrile illness, such as measles, influenza, meningitis,
erysipelas, psittacosis, Behçet disease
l. Chronic infections, such as tuberculosis or syphilis
m. Coccidioidomycosis
n. Parasitic infection, such as that due to teniae, Giardia,
Ancylostoma
o. Idiopathic
(2) Circinate retinopathy
a. Senile vascular disease
b. Venous obstruction
c. Diabetic retinopathy
d. Coats disease (retinal telangiectasia)
e. Retinal detachment
f. Anemia
g. Leukemia
h. Idiopathic (primary)
i. Retinal arterial macro aneurysm
(3) Diabetic retinopathy
5. Outer nuclear layer or photoreceptor elements
A. Congenital hereditary vision defects
(1) Trichromatism (anomalous)
(2) Dichromatism
(3) Monochromatism
B. Hereditary macular dystrophies
(1) Progressive cone dystrophy
(2) Inverse (macular) retinitis pigmentosa
C. Olivopontocerebellar degeneration
D. Light toxicity
(1) Operating microscope bum
(2) Solar bum
6. Pigment epithelium
A. Hereditary macular dystrophies
(1) Vitelliform dystrophy (Best disease)
(2) Adult onset foveomacular vitelliform dystrophy (adult Best)
(3) Fundus flavimaculatus
(4) Fundus flavimaculatus with macular involvement (Stargardt disease)
(5) Dominant drusen (Doyne honeycomb dystrophy)
(6) Reticular pigment dystrophy (Sjögren)
(7) Butterfly-shaped pigment dystrophy (Deutman)
(8) Central areolar choroidal and pigment epithelial dystrophy
(9) Sorsby fundus dystrophy
(1) Rubella syndrome (German measles)
(2) Acute posterior multifocal placoid pigment epitheliopathy
C. Toxic lesions
(1) Chloroquine
(2) Hydroxychloroquine
(3) Phenothiazine
a. Chlorpromazine
b. Thioridazine
(4) Sparsomycin
(5) Ethambutol
(6) Indomethacin
(7) Quinine
(8) Desferrioxamine
(9) Penicillamine
D. Drusen (senile, degenerative)
E. Refsum syndrome (phytanic acid storage disease)
F. Myotonic dystrophy syndrome
7. Bruch membrane
A. Angioid streaks associated with
(1) Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome)
(2) Senile elastosis of skin
(3) Osteitis deformans (Paget disease)
(4) Fibrodysplasia hyperelastica (Ehler-Danlos syndrome)
(5) Sickle cell anemia
(6) Acromegaly
(7) Beta-thalassemia
(8) Abetalipoproteinemia (Bassen-Kornzweig syndrome)
B. Lacquer cracks in pathologic myopia
C. Traumatic fracture of Bruch membrane
8. Pigment epithelium-Bruch membrane choriocapillaris
A. Degenerative lesions
(1) Disciform macular degeneration (senile, juvenile)
(2) Age-related macular degeneration
(3) Adult hereditary cerebromacular degeneration (Kufs?)
B. Serous detachment of neuroepithelium or pigment epithelium associated with
the following:
(1) Central serous chorioretinopathy
(2) Hemangioma of choroid
(4) Pit of the optic disk
(5) Hypotony (see p. 325-326)
(6) Leukemic infiltrates of choroid
(7) Terminal illness
(8) Trauma
(9) Uveitis
(10) Optic neuritis (see p. 578-585, 587-588)
(11) Papilledema (see p. 593-601)
(12) Acute hypertension
(13) Vitreous traction
(14) Angioid streaks (see p. 526-529)
(15) Vogt-Koyanagi-Harada syndrome
(16) Toxocara canis
(17) Myopic choroidal degeneration
(19) Choroidal nevus
(20) Collagen vascular disease
(21) Hemorrhagic or organized disciform detachment
9. Choroid
A. Degenerative lesions
(1) Central areolar choroidal atrophy
(2) Myopic choroidal atrophy
(3) Helicoid peripapillary chorioretinal atrophy (?)
(1) Histoplasmosis
C. Vascular occlusive lesions
10. Miscellaneous
A. Retinal inflammations (multilayer alterations that may involve the macula)
(1) Toxoplasma gondii
(2) T. canis
(3) Septic emboli
(4) Cytomegalovirus retinitis
(5) Candida organisms
(6) Bacteria
B. Congenital anomalies of the macula
(1) Aplasia
(2) Hypoplasia
(3) Heterotopia
(4) Colobomas (see p. 450)
(5) Aberrant macular vessels
Maumenee AE, Emery JM. An anatomic classification of diseases of the macula. Am J

Ophthalmol 1972; 74:594-599.
Yannuzzi LA, et al. The macula: a comprehensive text and atlas. Baltimore: Williams &
Wilkins, 1979.
BILATERAL MACULAR LESIONS
1. Development defects (colobomas)

allopurinol (?) clonidine (?) iodochlorhydroxyquin
amodiaquine griseofulvin (?) iodoquinol
broxyquinoline hydroxychloroquine quinine
chloroquine ibuprofen (?) thioridazine
chlorpromazine indomethacin (?)
3. Infectious entities
A. Herpes simplex
*B. Cytomegalic retinitis
C. Candidiasis and nocardiosis
D. T. canis (visceral larva migrans syndrome)
E. Congenital syphilis
*F. Tuberculosis
*G. Ocular histoplasmosis
H. Congenital toxoplasmosis
4. Intrauterine inflammations
5. Noninfectious entities
A. Best disease
B. Stargardt disease
*C. Exudative age-related macular degeneration
6. Presumed inflammatory origin
Bronstein MA, et al. Bilateral macular lesions. Ann Ophthalmol 1981; 13:859-861.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects. Woburn. MA:
PSEUDOMACULAR EDEMA
1. Exudative senile maculopathy-serous or hemorrhagic detachment of the macular retina

in persons 50 years of age or older, including "giant cyst of macula"
2. Serous detachment of retinal pigment epithelium
3. Central serous retinopathy caused by drugs, including the following:
adrenal cortex injection fludrocortisone paramethasone
aldosterone fluprednisolone prednisolone
betamethasone hydrocortisone prednisone
cortisone indomethacin triamcinolone (?)
desoxycorticosterone methylprednisolone
dexamethasone oral contraceptives
Fraunfelder FT. Fraunfelder FW. Drug-induced ocular side effects. Woburn, MA:
Gass JDM. Pathogenesis of disciform detachment of neuroepithelium. Am J Ophthalmol

1967; 63:573-711.
MACULAR EDEMA
This condition involves the loss of foveal depression with ophthalmoscope and
outline of multiple cystoid. Spaces can be retroilluminated with slit lamp; often a yellow
exudate lies deep within or beneath retina in foveal area.
1. Acquired parafoveal telangiectasis

2. Amebiasis (amebic dysentery)
3. Bang disease (brucellosis)
5. Carotid artery obstruction
6. Central angiospastic retinopathy
7. Coats disease
8. Choroidal tumors
9. Crohn disease
10. Cytomegalovirus retinitis
11. Diabetic retinopathy
12. Dominant inheritance macular dystrophy
acetazolamide aldosterone amithiozone
acetophenazine allopurinol (?) amodiaquine
adrenal cortex injection aluminum nicotinate aspirin
bendroflumethiazide fluprednisolone periciazine
benoxinate ganciclovir perphenazine
benzthiazide griseofulvin phenacaine
betamethasone hexamethonium phenylephrine (?)
betaxolol hyaluronidase piperacetazine
broxyquinoline hydrochlorothiazide polythiazide
bupivacaine hydrocortisone prednisolone
butacaine hydroflumethiazide prednisone
butaperazine hydroxychloroquine prilocaine
carbromal ibuprofen procaine
carphenazine indapamide prochlorperazine
chloramphenicol indomethacin (?) promazine
chloroquine iodide and iodine promethazine
chlorothiazide solutions and proparacaine
chlorpromazine compounds propiomazine
chymotrypsin (?) iodochlorhydroxyquin propoxycaine
cobalt (?) iodoquinol quinethazone
cortisone iothalamate quinine
cyclothiazide iothalamic acid radioactive iodides
desoxycorticosterone latanoprost sodium salicylate
dexamethasone levobunolol (?) sulindac
dibucaine lidocaine tamoxifen
dichlorphenamide meglumine and sodium tetracaine
diethazine meprednisone thiethylperazine
diiodohydroxyquin mesoridazine thiopropazate
dipivalyl epinephrine methazolamide thioproperazine
(DPE) methdilazine thioridazine
dipivefrin methotrimeprazine thiothalidomine
dyclonine methyclothiazide timolol
epinephrine methylprednisolone triamcinolone
ergot metisazone trichlormethiazide
estradiol naproxen trichloroethylene
ethambutol niacin trifluoperazine
ethopropazine niacinamide triflupromazine
ethoxzolamide nicotinic acid trimeprazine (?)
etidocaine nicotinyl alcohol zidovudine
fludrocortisone paramethasone
fluphenazine perazine
14. Electrical injuries to the retina

15. Epikeratophakia complication
16. Fabry disease (ceramide trihexoside lipidosis)
17. Felty syndrome (rheumatoid arthritis with hypersplenism)
18. Following corneal-relaxing incisions
19. Goldmann-Favre disease (hyaloideoretinal degeneration)
20. Gyrate atrophy
22. Hemangiomas of choroid
23. Hemangioma of choroid
24. Hunter syndrome (mucopolysaccharidoses II, or MPS II)
26. Hypertensive retinopathy
27. Hypotony (postoperative)
*28. Irvine-Gass syndrome
29. Large central foveal cyst
30. Leukemia
31. Meningococcemia (Neisseria meningitidis)
32. Macular dystrophy-dominant
33. Nematode, intraretinal
34. Nylon suture toxicity
35. Optic nerve pit
36. Pars planitis (peripheral uveitis)
37. Photocoagulation
38. Porphyria cutanea tarda
39. Posterior capsule rupture
*40. Preretinal fibrosis (macular pucker)
41. Punctata albescens retinopathy
42. Radiation retinopathy
44. Retinohypophysary syndrome (Lijo-Pavia-Lis syndrome)
45. Scheie syndrome
46. Scleral buckle
47. Silverman syndrome (battered baby syndrome)
48. Subacute sclerosing panencephalitis (Dawson disease)
49. Toxoplasmic chorioretinitis
50. Trauma to globe (commotio retinae)
51. Ultraviolet light from sun, operating microscope, or other bright light sources
52. Uveitis (anterior or posterior)
*53. Vein occlusion, including branch vein occlusion (see p. 468)
55. Yttrium-aluminum-garnet (YAG) laser posterior capsulotomy
Axer-Siegel R. et al. Cystoid macular edema after cataract surgery with intraocular
vancomycin. Ophthalmology 1999; 106; 1660-1664.
Miyake K, et al. Latanoprost accelerates disruption of the blood-aqueous barrier and the
incidence of angiographic cystoid macular edema in early postoperative pseudophakias.
Moroi SE, et al. Cystoid macular edema associated with latanoprost therapy in a case
series for patients with glaucoma and ocular hypertension. Ophthalmology 1999;
106:1024-1029.
Pinckers A, et al. Colour vision in retinitis pigmentosa: influence of cystoid macular

edema. Int Ophthalmol 1993; 17:143-146.
Williams & Williams, 2002.
Warwar RE. Cystoid macular edema and anterior uveitis associated with latanoprost use.
Opthalmology 1998; 105: 263-266.
ABSENCE OF FOVEAL REFLEX

The absence of foveal reflex is caused by drugs, including amodiaquine,
chloroquine, diiodohydroxyquin, hydroxychloroquine, iodochlorhydroxyquin, or quinine.
Butterworth-Heinemann. 2001.
MACULAR PUCKER
Macular pucker involves tiny folds that often are arranged in a stellate manner
around macula and usually are associated with a preretinal membrane (preretinal macular
fibrosis, preretinal vitreous membrane, surface wrinkling retinopathy, cellophane
maculopathy).
1. Associated with proliferative retinopathy

*A. Diabetes retinopathy
B. Eales disease
*C. Hypertension
D. Sickle cell disease
E. Vein occlusion
2. Central serous chorioretinopathy
A. Psychopharmacologic medication use
B. Corticosteroid use
C. Hypertension
3. Congenital
4. Following photocoagulation or cryoretinopexy
5. Following traumatic posterior vitreous separation, such as blunt trauma to the eye and
whiplash injury (craniocervical syndrome)
6. Loss of formed vitreous at operation
7. Idiopathic (probably related to spontaneous posterior vitreous detachment)
8. Macular detachment
9. Multiple retinal operations
10. Penetrating or blunt injuries
11. Posterior uveitis
* 12. Proliferative vitreoretinopathy following vitreoretinal surgery
14. Trauma (blunt)
McDonald HR, et al. Surgical management of idiopathic epiretinal membranes.

Smiddy WE, et al. Clinicopathologic study of idiopathic macular pucker in children and
young adults. Retina 1992; 12:232-236.
Tittl MK, et al. Systemic findings associated with central serous chorioretinopathy. Am J
Ophthalmol 1999; 128: 63-68.
Uemura A, et al. Macular pucker after retinal detachment surgery. Ophthalmic Surg
Lasers 1992; 23:116-119.
MACULAR EXUDATES AND HEMORRHAGES
1. Retinal macroaneurysms
2. Hemorrhagic age-related macular degeneration (ARMD)
3. Diabetic macular edema
4. Retinal telangiectasis
5. With dense subretinal and subretinal pigment epithelium (sub-RPE [retinal pigment
epithelium]) hemorrhage simulate the appearance of retinal macroaneurysms.
Wilkins, 1979.
MACULAR STAR OR STELLATE RETINOPATHY (EXUDATES IN A STAR

FORMATION RADIATING AROUND MACULA IN THE NERVE FIBER
LAYER)
1. Acute febrile illness, such as measles, influenza, meningitis, erysipelas, psittacosis,

Behçet disease (dermatostomatoophthalmic syndrome)
2. Cat-scratch fever
3. Chronic infections, such as tuberculosis or syphilis
5. Gansslen syndrome (familial hemolytic icterus)
*6. Hypertension
7. Idiopathic
8. Juxtapapillary choroiditis (Jensen disease)
*9. Neuroretinitis
10. Obstruction of the artery or vein supplying the macular area (see p. 457 and 468)
11. Ocular or cerebral trauma
12. Parasitic infection, such as that due to teniae, Giardia, Ancylostoma
*13. Papilledema (see p. 563-601)
14. Papillitis (see p. 578-585, 587-588)
15. Retinal periphlebitis
Wilkins, 1979.
Extracted Table Stellate Retinopathy
RETINOCILIARY VEIN-DISAPPEARS FROM THE RETINA AT DISC

MARGIN WITHOUT CONNECTION TO CENTRAL RETINAL VEIN
1. Acquired
A. Arachnoid cyst of the optic nerve
B. Central retinal vein occlusion (see p. 468)
C. Chronic atrophic papilledema from causes including craniopharyngioma (see
p. 595-597)
D. Glioma of the optic disc
2. Congenital
Wolter JJ. Retinociliary vein associated with a craniopharyngioma. Ann Ophthalmol

1979; 11:751.
CHERRY-RED SPOT IN MACULA (RULE OUT MACULAR HEMORRHAGE)
1. Cardiac myxomas
2. Cryoglobulinemia
3. Dapsone poisoning
4. Hallervorden-Spatz disease (pigmentary degeneration of globus pallidus)
*7. Hypertension (severe)
8. Intralesional chalazion corticosteroid injection
10. Macular hemorrhage
* 11. Macular hole with surrounding retinal detachment
12. ML I (lipomucopolysaccharidosis)
*15. Occlusion of central retinal artery (see p. 457)
16. Quinine toxicity
17. Sphingolipidoses
A. Cherry-red spot myoclonus
B. Farber syndrome (Farber lipogranulomatosis)
C. Gangliosidosis GM1-type (juvenile gangliosidosis)
D. Gaucher disease (glucocerebroside storage disease)
E. Goldberg syndrome
F. Infantile metachromatic leukodystrophy (van Bogaert-Nyssen disease)
G. Niemann-Pick disease type A
H. Niemann-Pick disease type B
I. Sandhoff disease (gangliosidosis GM2-type 2)
*J. Tay-Sachs disease (gangliosidosis GM-type I)
18. Steroid injection intranasally
*19. Temporal arteritis (giant cell arteritis)
20. Traumatic retinal edema (commotio retinae; Berlin edema)
21. Vogt-Spielmeyer cerebral degeneration (Batten-Mayou syndrome)
Abhayambika K, et al. Peripheral neuropathy and haemolytic anaemia with cherry red
spot on macula in dapsone poisoning. J Assoc Physicians India 1990; 38:564-565.
Reed JB. et al. Bartonella henselae neuroretinitis in cat scratch disease. Ophthalmology
1998; 105:459-466.
Wade NK. et al. Optic disk edema associated with peripapillary serous retinal
detachment: an early sign of systemic Bartonella henselae infection. Am J Ophthalmol
2000; 130:327-334.
MACULAR HEMORRHAGE
1. Choroidal neovascular membranes

*A. Age-related macular degeneration
B. Angioid streaks
C. Histoplasmosis
D. Idiopathic
E. Pathologic myopic
F. Posterior uveitis
2. Infectious retinitis
*A. Cytomegalovirus retinitis
B. Subacute bacterial endocarditis
3. Retinal vascular disease
A. Radiation retinopathy
B. Retinal arterial macro aneurysm
*C. Vein occlusion
A. Blood dyscrasias
(1) Anemia
(2) Leukemia
(3) Polycythemia vera
(4) Sickle cell disease
(5) Thrombocytopenia
(6) Waldenström macroglobulinemia
B. Cardiovascular shock (especially gastrointestinal hemorrhage)
*C. Diabetes mellitus
*D. Human immunodeficiency virus (HIV)-related retinopathy
*E. Hypertension
F. Toxemia of pregnancy
5. Trauma
A. Choroidal rupture
B. Purtscher retinopathy
C. Shaken-baby syndrome
D. Terson syndrome
E. Valsalva retinopathy
F. Vitreous detachment
McCabe CM, et al. Nonsurgical management of macular hemorrhage secondary to retinal

artery macroaneurysms. Arch Ophthalmol 2000; 118:780-786.
Paris CL, et al. Neonatal macular hemorrhage. Int Ophthalmol 1991; 15:153-155.
Stevenson A, et al. Is aspirin a factor in macular hemorrhage. Ophthalmol Times 1993;

18:32-34
Extracted Table Macular Hemorrhage
PARAFOVEAL TELANGIECTASIA
This condition is a retinal microvascular anomaly involving the parafoveal
capillary network as well as the immediately adjacent vascular bed and is best
demonstrated by fluorescein angiography.
1. Carotid artery obstruction

*2. Diabetes mellitus, usually bilateral
*3. Idiopathic
4. Localized form of Coats disease, usually unilateral
*5. Small branch vein occlusion
6. Small retinal capillary hemangioma, usually unilateral
7. Roentgenogram, irradiation
Gass JD, Oyakawa T. Idiopathic juxtafoveal telangiectasia. Arch Ophthalmol 1982;

100:769.
Millay RH. et al. Abnormal glucose metabolism and parafoveal telangiectasia. Am J

Ophthalmol 1986:102:363-370.
MICROHEMORRHAGIC MACULOPATHY -SMALL MONOCULAR
MACULAR HEMORRHAGE THAT IS PUNCTATE, ROUND OR BILOBED
1. Increased venous stasis (Valsalva stress)

2. Impaired blood platelet aggregation
3. Medications that impair platelet function including aspirin, ibuprofen (Motrin),
pentazocine, propranolol hydrochloride and oral contraceptives.
Pruett RC. Carvalho ACA, Trempe CL Microhemorrhagic maculopathy. Arch

MACULAR CYST
Macular cyst must be differentiated from macular hole with Hruby lens or contact
lens and slit lamp.
1. Amebiasis
2. Cysticercosis-subretinal cyst
*3. Cystic degeneration-common following trauma, uveitis, and vascular disease
4. Hamman-Rich syndrome (alveolar capillary block syndrome)
5. Histoplasmosis
6. Hydatid disease (echinococcosis)
7. Parasitic and mycotic cysts
McDonnell PJ. et al. Clinical features of Idiopathic macular cysts and holes. Am J
Ophthalmol 1982:93:777-786.
MACULAR HOLE
Macular hole must be differentiated from macular cyst with Hruby lens or contact
lens and slit lamp)
* 1. Idiopathic (most common, may be bilateral)

2. From the following:
A. Edema (see p. 439)
(1) Inflammatory
(2) Toxic
(3) Vascular
(4) Following papilledema
B. High myopia
C. Ischemic, such as with retinal detachment or choroidal tumor-the macula is
separated from choriocapillaris
D. Degenerative conditions of the retina and retinal dystrophy
E. Trauma
F. Radiation injury
G. Glaucoma
H. Posterior senile retinoschisis
I. High tension electric shock
J. Central serous chorioretinopathy
K. Optic disc coloboma
L. Posterior retinal detachment associated with optic pits
M. Industrial laser bums
N. Lightening-induced
O. Posterior microphthalmos
P. Septic embolization
Q. Subhyaloid hemorrhage
R. Topical pilocarpine use
S. YAG laser
4. Foveomacular retinitis-usually young males
*5. Pseudohole due to epiretinal membrane (may differentiated from true hole by
fluorescein angiography)
6. Sickle cell disease
Benedict WL, Shami M. Impending macular hole associated with topical pilocarpine. Am
J Ophthalmol 1992; 114: 765-779.
Blacharski PA, Newsome DA. Bilateral macular holes after Nd:YAG laser posterior
capsulotomy. Am J Ophthalmol 1988; 105:451-459.
Gass JDM. Idiopathic senile macular hole. Arch Ophthalmol 1988; 106:629-639.
Ho AC, et al. Unusual immunogammopathy maculopathy. Ophthalmology 2000;

107:1099-1103.
Lansing MB, et al. The effect of pars plana vitrectomy and transforming growth factor-
beta without epiretinal membrane peeling on full-thickness macular holes.
Smiddy WE. Atypical presentations of macular holes. Arch Ophthalmol 1993; 111:626-
631.
MACULAR COLOBOMA
This condition involves bilaterally symmetric, circumscribed, excavated defects in
choroid and retina in the region of macula associated with reduced vision
1. Autosomal dominant
2. Autosomal recessive inheritance with skeletal anomalies
3. Conditions that exhibit choroidal coloboma (see p. 555-556)
4. Down syndrome
5. Hypercalciuria, myopia, and macular coloboma
6. Isolated
7. Macular coloboma with brachydactyly
8. Sorsby syndrome I
Yamaguchi K, Tamai M. Congenital macular coloboma in Down syndrome. Ann

Ophthalmol 1990; 22:222-223.
ELEVATED MACULAR LESION
1. Angiospastic retinopathy
2. Central serous detachment of retina
3. Chorioretinitis especially histoplasmosis and toxoplasmosis
4. Choroidal hemangioma
7. Varix of the vortex ampulla
8. Sickle cell disease
AI-Abdulla NA, et al. Sickle cell disease presenting with extensive peri-macular
arteriolar occlusions in a nine-year-old boy. Am J Ophthalmol 2000; 130:419-428.
Cunningham ET, et al. Central serous chorioretinopathy in patients with systemic lupus
erythematosus. Ophthalmology 1996; 103:2081-2090.
1991.
HETEROTOPIA OF THE MACULA

This condition involves an abnormal location of the macula in relation to the optic
disc; the eye with the ectopic macula tends to deviate in the same direction as macular
displacement; visual fields show displacement of blind spot and cover-uncover test shows
no shift of fixation.
1. Chorioretinitis
2. Congenital
*3. Retinopathy of prematurity
4. Inflammatory
*5. Proliferative diabetic retinopathy
Bresnick GH. Visual function abnormalities in macular heterotopia caused by

proliferative diabetic retinopathy. Am J Ophthalmol 1981; 92:85-102.
Stem SD, Arenberg IK. Heterotopia of the macula with associated retinal detachment. J
Pediatr Ophthalmol 1969; 6:198-202.
WHITE OR YELLOW FLAT MACULAR LESION AND PIGMENTARY
CHANGE
1. Posttraumatic - pigmentary disturbance; cysts or hole at macula

2. Postinflammatory - chorioretinal atrophy with pigment clumping at center and
periphery of lesion
3. Coloboma of macula-atrophic area at macula often associated with coloboma of disc;
sclera may be ectatic (see p. 450)
4. Radiation injuries-common after solar eclipse; punched-out appearance
5. Fuchs dark spot-pigmented spot associated with other signs of degenerative myopia
adrenal cortex injection dexamethasone (?) iodochlorhydroxyquin
aldosterone diiodohydroxyquin methylprednisolone
allopurinol (?) fludrocortisone oral contraceptives
amodiaquine fluprednisolone (?) paramethasone (?)
betamethasone (?) griseofulvin prednisolone (?)
chloroquine hydrocortisone (?) prednisone (?)
cortisone (?) hydroxychloroquine quinine
desoxycorticosterone (?) indomethacin (?) triamcinolone
7. Stellate retinopathy - star-shaped exudates (see p. 443)

8. Hard exudates and circinate retinopathy (see p. 495)
9. Drusen – common, discrete yellow spots beneath the retina
10. Doyne honeycomb choroiditis-rare; honeycomb pattern of yellow patches at posterior
pole; degenerative changes at macula
11. Heredomacular dystrophies
A Best disease (vitelliruptive macular dystrophy) up to 18 years of age; egg-yolk
lesion at macula, later absorbed to leave atrophic scar
B. Fundus flavimaculatus - yellow patches at posterior pole; degenerative changes
at macula
C. Stargardt disease (juvenile macular degeneration) to 10 years of age; variable
appearance in different families; bilateral lesions showing some degree of
symmetry
D. Behr disease (optic atrophy-ataxia syndrome) – adults, similar to Stargardt
type
E. Presenile and senile-pigmentary changes followed by atrophy, bilateral and
symmetric
13. Central choroidal sclerosis - rare, atrophic retina with sclerosed choroidal vessels
showing clearly
14. Central areolar choroidal atrophy-rare, exudate and edema followed by sharply
defined atrophic area with white strands of choroidal vessels
15. Pseudoinflammatory macular dystrophy-rare, initially edema and exudates followed
by scarring with pigmentary disturbance and atrophic patches
16. Gaucher disease (glucocerebroside storage disease)-rare, ring-shaped macular lesions,
lipid deposits in cornea and conjunctiva
17. Diffuse leukoencephalopathy - rare, white deposits in periphery and macular area
18. Sjögren-Larsson syndrome (oligophrenia-ichthyosis-spastic diplegia syndrome)
19. Angioid streaks (see p. 526-529)
20. Multiple evanescent white-dot syndrome (MEWDS) usually unilateral, predominantly
healthy women, vitreitis
21. Acute multifocal placoid pigment epitheliopathy – rare, map-like pigmentary
disturbance of posterior pole or more widespread over posterior fundus
Eagle RC, et al. Retinal pigment epithelial abnormalities in fundus flavimaculatus.

Ophthalmology 1980:87:1189.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects and drug interactions.
Woburn, MA: Butterworth- Heinemann, 2001.
Hadden OB, Gass DM. Fundus flavimaculatus and Stargardt's disease. Am J Ophthalmol
1976; 82:527-539.
PIGMENTARY CHANGES IN MACULA
1. Hereditary macular degeneration without cerebral or other disease

A Best disease (vitelliform macular dystrophy)
B. Stargardt disease (juvenile flavimaculatus)
C. Behr syndrome (optic atrophy-ataxia syndrome)
*2. Retinitis pigmentosa
3. Secondary pigmentary retinopathy following trauma or inflammation (see p. 497)
*4. Age-related macular degeneration
5. Metabolic disease associated with pigmentary retinopathy
A. Abetalipoproteinemia (Bassen-Kornzweig syndrome)
B. Alpha-lipoprotein deficiency (Tangier syndrome)
C. Ceroid lipofuscinosis
(1) Batten-Mayou syndrome
(2) Dollinger-Bielschowsky syndrome, late infantile (Bielschowsky-
Jansky disease)
(3) Infantile type of neuronal ceroid lipofuscinosis
D. Hepatic disease
E. Refsum disease (phytanic acid storage disease)
F. Tay-Sachs syndrome (gangliosidosis GM2-type I)
G. Vitamin A
H. MPS
(1) Hunter syndrome (MPS II)
(2) Hurler syndrome (MPS I-H)
(3) Sanfilippo-Good syndrome (MPS III)
(4) Scheie syndrome (MPS I-S)
*6. Drugs, including the following:
acetophenazine amodiaquine benzatropine (?)
amiodarone (?) azathioprine biperiden (?)
butaperazine chlorpromazine perazine
carbamazepine chlorprothixene pericyazine
carphenazine cisplatin perphenazine
cefaclor clofazimine piperacetazine
cefadroxil clonidine (?) prazosin (?)
cefamandole cobalt (?) prochlorperazine
cefazolin cycrimine (?) procyclidine (?)
cefonicid deferoxamine promazine
cefoperazone diethazine promethazine
ceforanide diethylcarbamazine propiomazine
cefotaxime ethambutol (?) quinacrine (?)
cefotetan ethopropazine quinine
cefoxitin fluphenazine sulindac (?)
cefsulodin hydroxychloroquine tamoxifen
ceftazidime indomethacin (?) thiethylperazine
ceftriaxone ketoprofen (?) thiopropazate
cefuroxime mesoridazine thioproperazine
cephalexin methdilazine thioridazine
cephaloridine methotrexate thiothixene
cephalothin methotrimeprazine trifluoperazine
cephapirin minoxidil (?) triflupromazine
cephradine mitotane trihexyphenidyl (?)
chloramphenicol moxalactam trimeprazine
chloroquine naproxen
chlorphenoxamine (?) penicillamine
7. Inflammation
*A. Toxoplasmosis
B. Trauma
8. Multifocal necrotizing encephalopathy
10. Dialinas-Amalric syndrome (deaf mutism-retinal degeneration syndrome)
Fishman GA, et al. X-linked recessive retinitis pigmentosa. Arch Ophthalmol 1986;
104:1329-1335.
BULL'S-EYE MACULAR LESION-CIRCULAR AREA OF RETINAL PIGMENT

EPITHELIUM ATROPHY SURROUNDING A SPARED FOVEA
1. Autosomal-dominant, benign, concentric annular macular dystrophy
2. Ceroid lipofuscinosis
*3. Chloroquine or hydroxychloroquine retinopathy
4. Cone dystrophy
5. Hereditary ataxia
6. NARP syndrome
7. Spielmeyer-Vogt syndrome (Batten-Mayou syndrome)
*8. Stargardt disease (or fundus flavimaculatus)
9. Trauma
10. Unknown
Duinkerke-Eerola, et al. Atrophic maculopathy associated with hereditary ataxia. Am J

Isenberg SJ. The eye in infancy. Chicago: Year Book Medical. 1989.
Nomura R, et al. Unilateral cone dysfunction with bull's eye maculopathy.

MACULAR WISPS AND FOVEOLAR SPLINTER

These are noted in focal illumination with Goldmann contact lenses but are
invisible ophthalmoscopically.
1. Direct and indirect ocular concussion

2. Following absorption of small prefoveal hemorrhage
3. Foveomacular retinitis
4. Juvenile macular degeneration
5. Old, healed chorioretinitis
7. Spontaneous senile posterior vitreous detachment
8. Whiplash injury
Daily L. Foveolar splinter and macular wisps. Arch Ophthalmol 1970; 83:406-411.
Daily L. Further observations on foveolar splinter and macular wisps. Arch Ophthalmol
1973; 90:102-103.
MACULAR HYPOPLASIA (INCOMPLETE MACULAR DEVELOPMENT

MANIFESTED BY DECREASED VISION)
* 1. Albinism
*2. Associated with autosomal-dominant aniridia
3. Associated with microcornea and corectopia
4. Associated with myelinated nerve fibers
5. Forsius-Eriksson syndrome (Aland disease)
*6. Goldenhar-Gorlin syndrome (oculoauriculovertebral dysplasia)
7. Krause syndrome (encephaloophthalmic dysplasia)
8. Ring chromosome
9. Syndrome of foveal hypoplasia and presenile cataract (O'Donnell-Pappas syndrome) -
autosomal dominant
10. Tuomaala-Haapanen syndrome
Ghose S, Mehta U. Microcornea with corectopia and macular hypoplasia in a family. Jpn
J Ophthalmol 1984; 28: 126-130.
Margolis S, et al. Retinal and optic nerve findings in Goldenhar-Gorlm syndrome.

Ophthalmology 1984;91: 1327-1333.
Roy FH. Ocular syndromes and systemic diseases, 3rd ed Philadelphia: Lippincott
Szymanski KA, et al. Genetic studies of ocular albinsm in a large Virgina kindred. Ann
Ophthalmol 1984;16: 183-185.
PREMACULAR SUB HYALOID HEMORRHAGE
1. Branch retinal vein occlusion

2. Blood dyscrasia
4. Retinal macroaneurysm
5. Terson syndrome
6. Valsalva retinopathy
Ulbig MW, et al. Long-term results after drainage of premacular subhyaloid hemorrhage
into the vitreous with a pulsed Nd: YAG laser.
RETINAL VASCULAR TORTUOSITY
1. Acute malnutrition
2. Aortic coarctation
3. Bazzana syndrome (angiospastic ophthalmoauricular syndrome)
4. Choked disc (see p. 593)
5. Chronic respiratory insufficiency, such as in cystic fibrosis and familial dysautonomia
(Riley-Day syndrome)
7. Congenital
9. Cryoglobulinemia
10. Down syndrome (trisomy 21)
*14. Glaucoma, open angle
15. Granulocytic sarcoma of orbit
16. Hereditary hemorrhagic telangiectasis (Osler disease)-tortuosity and varicosity
17. Hypertension
18. Kenny syndrome (dwarfism, thickened long bone cortex, transient hypocalcemia)
19. Leukemia
20. Lymphogranuloma venereum (Nicolas-Favre disease)
21. Maroteaux-Lamy syndrome (mucopolysaccharidoses type VI)
22. Macroglobulinemia
23. Mosse syndrome (polycythemia-hepatic cirrhosis syndrome)
24. Myopia
25. Normal variation with fullness
26. Polycythemia with vessel fullness
28. Racemose hemangioma of retina, angiomatosis retinae without obvious tumor
formation, or von Hippel-Lindau syndrome (retinocerebral angiomatosis)
*30. Sickle cell disease
31. Visceral larva migrans (nematode ophthalmia syndrome)
Davis JL, et al. Granulocytic sarcoma of the orbit. Ophthalmology 1985; 92:1758-1762.
Wells CG, Kalina RE. Progressive inherited retinal arteriolar tortuosity with spontaneous
retinal hemorrhages. Ophthalmology 1985; 92: 1015-1024.
VENOUS BEADING

2. Loaiasis (Loa loa)
3. Macroglobulinemia (Waldenstrom syndrome)
Schlaegel TF. Essentials of uveitis. Boston: Little, Brown. 1969.
OPHTHALMODYNAMOMETRY
When blood pressure of the retinal artery is measured, a difference between the
eyes of about 15% of diastolic readings is considered significant.
1. False-positive or variable readings

A. Abnormally high or low intraocular pressure or asymmetry between the two
eyes
B. Cardiac abnormalities, such as atrial fibrillation, heart block, or extrasystoles
C. Marked asymmetry of retinal vessels in the two eyes
D. Measurements of ophthalmic artery pressure lower than 20 g on the instrument
E. Poor patient cooperation
F. Variation in systemic blood pressure between readings
2. High ophthalmodynamometry values
A. Basilar-vertebral occlusion
B. Bilateral distal internal carotid occlusion-unusual
C. Progressive intracranial arterial occlusion syndrome
3. Low ophthalmodynamometry values
A. Both sides reduced with orthostatic hypotension
*B. Reduced on side of an occluded internal carotid artery
Wilkins, 1985.
PULSATION OF RETINAL ARTERIOLE (HIGH PULSE PRESSURE)
1. Aortic regurgitation
2. Hyperthyroidism
*3. Intraocular blood pressure higher than diastolic blood pressure but lower than systolic
blood pressure
Newell FW. Ophthalmology: principles and concepts. 7th ed. St. Louis: CV Mosby.
1992.
RETINAL ARTERY OCCLUSION

This condition involves a sudden, painless visual loss; on ophthalmoscopic
examination, a diffuse retinal pallor and a cherry-red spot in macula are noted.
1. Embolism-cardiac or pulmonary sources

A. Air emboli following trauma or surgery
B. Amniotic fluid embolization
C. Cardiac myxoma
D. Corticosteroid emboli
E. Espildora-Luque syndrome (ophthalmic Sylvian syndrome)
F. Fat emboli following long-bone fractures
G. Iatrogenic trauma induced by angiography
*H. In older patients-due to atheroma of carotid artery
I. In young persons-due to postrheumatic vegetations (rheumatic fever), cardiac
catheterization, or valvotomy
J. Leudoemboli - vasculitis, Purtscher retinopathy, septic endocarditis
K. Moyamoya disease (multiple progressive intracranial arterial occlusion)
L. Nicolau syndrome (emboli of medication inadvertently introduced into artery)
M. Synthetic material used in cardiac and vascular procedures
N. Talc emboli-long-term intravenous drug abusers
O. Tumors-atrial myxoma, mitral valve papillary fibroelastoma
P. With cerebral infarction after periocular subcutaneous cosmetic silicone
injection
*2. Atherosclerosis of common carotid artery (ophthalmodynamometry employed for
diagnosis)
3. Ischemia
A. Carotid occlusion or dissection
B. Essential hypotension
C. Following orbital floor fractures or repair
D. Following surgery for retinal detachment
E. Generalized shock
F. Heart failure (rare)
G. Kahler disease (multiple myeloma)
H. Knee-chest position
I. Massive hemorrhage, such as that occurring in hematemesis. Gastrointestinal
bleeding, or surgical procedures
J. Migraine
K. Mosse syndrome (polycythemia-hepatic cirrhosis syndrome)
L. Orbital hemorrhage following retrobulbar injection
M. After surgery for scoliosis
N. Too rapid lowering of blood pressure in hypertensive subjects
4. Inflammation
A. Abdominal typhus (typhoid fever)
B. African eye-worm disease (loiasis)
C. Arteriole vasculitis, such as periarteritis nodosa (Kussmaul disease)
D. Bacterial endocarditis
E. Behçet disease (dermatostomatoophthalmic syndrome)
F. Diphtheria
G. Familial factor V Leiden polymorphism and positive rheumatoid factor
H. Giant cell arteritis
I. Herpes zoster
J. Metastatic bacterial endophthalmitis
K. Mucormycosis (phycomycosis)
L. Pancreatitis
M. Recurrent toxoplasmic retinochoroiditis
N. Rocky Mountain spotted fever (spotted fever)
O. Rubeola (measles)
P. Subacute bacterial endocarditis
Q. Systemic lupus erythematosus
R. Takayasu disease (pulseless disease)
*S. Temporal arteritis
T. Toxoplasma retinochoroiditis
U. Varicella (chickenpox)
5. Blood disease
A. After platelet transfusion
B. Following ocular trauma with secondary glaucoma in youths with sickle-trait
hemoglobinopathy
C. Polycythemia vera (Vaquez-Osler syndrome)
D. Sickle cell disease
7. Associated factors
8. Diathermy of persistent hyaloid
A. Drusen of optic nerve (see p. 559-560)
B. Giant cell arteritis
C. Papilledema (see p. 593-601)
D. Subdural cerebral hemorrhage
E. Arteriosclerosis of central retinal artery
F. Chronic simple glaucoma
9. After dye, yellow photocoagulation
10. Complication of retrobulbar block
11. Degos syndrome (malignant atrophic papulosis)
12. Disseminated lupus erythematosus
13. Fabry-Anderson syndrome (glycosphingolipid lipidosis)
14. Goldenhar-Gorlin syndrome (oculoauriculovertebral dysplasia)
16. Hyperhomocystinemia
17. Lyme disease
18. Neoplastic angioendotheliomatosis
20. Protein S deficiency
22. Sneddon syndrome (livedo reticularis, neurologic abnormalities, and labile
hypertension)
23. Use of tranexamic acid therapy
Dori D, et al. Multiple retinal arteriolar occlusions associated with coexisting primary
antiphospholipid syndrome and factor V Leiden mutation. Am J Ophthalmol 2000;
129:106-108.
Friedberg MA, Micale AJ. Monocular blindness from central retinal artery occlusion
associated with chickenpox. Am J Ophthalmol 1994; 117: 117-118.
Grossman W, Ward WT. Central retinal artery occlusion after scoliosis surgery with a
horseshoe headrest. Spine 1993; 18: 1226-1228.
Rao TH, et al. Central retinal artery occlusion from carotid dissection diagnosed by
cervical computed tomography. Stroke 1994; 25:1271-1272.
Russell SR, Folk JC. Branch retinal artery occlusion after dye yellow photocoagulation of
an arterial macroaneurysm. Am J Ophthalmol 1987; 104:186.
Extracted Table Retinal artery occlusion
LOCALIZED ARTERIAL NARROWING
1. Retinal atrophy following:

A. Degeneration
B. Inflammation
C. Trauma
D. Treatment with diathermy, light, or cryopexy
2. Any vascular retinopathy
Nover A. The ocular fundus: methods of examination and typical findings, 4th ed.
Philadelphia: Lea & Febiger, 1981.
Perkins ES, Dobree JH. The differential diagnosis of fundus conditions. St. Louis: CV
Mosby, 1972.
GENERALIZED ARTERIAL NARROWING
1. Local causes
A. Apparent narrowing
(1) High hypermetropia-common, small disc, narrow vessels, sometimes
pseudopapilledema (see p. 601)
(2) Congenital microphthalmos - rare, hypermetropia, often cataract (see
p. 252-253)
(3) Aphakia - cataract operation, dislocated lens (see p. 405)
(4) Hollenhorst syndrome (chorioretinal infarction syndrome)
(5) Wagner syndrome (hyaloideoretinal degeneration)
B. Trauma
(1) Avulsion of optic nerve-rare, secondary optic atrophy
(2) Fracture involving bony optic canal-rare, secondary optic atrophy
(3) Following retroocular injection-rare secondary optic atrophy
(4) Orbital hemorrhage following retroocular injection or orbital
operation- rare, secondary optic atrophy
(5) Carotid ligation for carotid-cavernous fistula, rare, secondary optic
atrophy
(6) Following angiography-rare, secondary optic atrophy
(7) Siderosis bulbi-metallic intraocular foreign body
C. Infection and edema
(1) Orbital cellulitis - exophthalmos, restricted ocular movements
(2) Following thyrotropic exophthalmos-ocular muscle paresis, lid
retraction
D. Degenerations, such as progressive cone-rod degeneration
E. Primary tapetoretinal degenerations, such as retinitis pigmentosa; Hallgren
syndrome (retinitis pigmentosa-deafness-ataxia syndrome)
2. Systemic disease
A. Arteriosclerosis
(1) In involutionary sclerosis-population older than 50 years of age,
generalized arteriolar narrowing, diminished light reflexes
(2) In arteriosclerotic disease
a. Arteriosclerotic central artery occlusion-common arteriovenous
crossing signs, focal arteriolar constriction
*b. Embolus from atheromatous plaque, common, sudden onset,
visible white embolus
B. Hypertensive conditions
*(1) Essential hypertension-retinal hemorrhages, cotton-wool spots,
arteriovenous crossing signs
*(2) Malignant hypertension-retinal hemorrhages, cotton-wool spots,
edema of disc
(3) Toxemia of pregnancy-rare, sometimes hemorrhages, cotton-wool
spots, edema of disc, serous detachment
(4) Coarctation of aorta-rare, hypertensive changes vary greatly in degree
(5) Pheochromocytoma-rare, hypertensive changes vary greatly in degree
(6) Adrenal tumor, hyperaldosteronism (adrenal medulla tumor syndrome)
- rare, hypertensive changes vary greatly in degree
(7) Cushing tumor (adrenocortical hyperfunction)-rare, hypertensive
changes vary greatly in degree
(8) Motor-neuron disease of cervicothoracic cord hypertension; may occur
after prolonged artificial pulmonary ventilation
C. Other forms of vascular disease
(1) Retinal ischemia -hypotension following severe or recurrent bleeding,
unilateral blindness in patients
*(2) Temporal arteritis (cranial arteritis, giant -cell arteritis)-common, 50
years or older; mean age at onset, 55 years; sudden blindness at onset
(3) Polyarteritis nodosa (Kussmaul disease)-multiple signs involving
choroid, retina, cornea, episclera, and ocular muscles
(4) Proliferative diabetic retinopathy-arterial narrowing occurs in 17% of
patients with proliferative diabetic retinopathy, mainly in cicatricial stage
(5) Cardiac arrest-thread-like arterioles, segmentation of blood column,
generalized retinal pallor, pallor of disc, sometimes macular cherry-red
spot
(6) Raynaud disease (idiopathic paroxysmal digital cyanosis)-young
adults, more common in women
D. Renal disease
(1) Acute glomerulonephritis-preceding illnesses, including scarlet fever,
streptococcal tonsillitis, otitis media, erysipelas (St. Anthony fire),
subacute bacterial endocarditis, polyarteritis nodosa (Kussmaul disease)
(2) Chronic glomerulonephritis-often asymptomatic and found on routine
examination
(3) Pyelonephritis and pyelitis-most common causes of renal failure
E. Diseases of the central nervous system
(1) Migraine
*(2) Syphilitic neuroretinitis
(3) Viral neuroretinitis (rare complication)
(4) Tay-Sachs disease (amaurotic familial idiocy)
(5) Jansky-Bielschowsky disease (amaurotic familial idiocy, late form)
(6) Myotonic dystrophy syndrome (Curschmann-Steinert syndrome)
(7) Retinohypophysis syndrome (Lijo Pavia-Lis syndrome)
(8) Zellweger syndrome (cerebrohepatorenal syndrome of Zellweger)
F. Toxic causes
(1) Chloroquine, hydroxychloroquine, quinacrine, amodiaquine
(2) Lead
(3) Quinine - rare, may follow large dose (abortifacient) or normal dose in
sensitive subjects
G. Other causes
(1) Caisson syndrome (bends)
(2) Chédiak-Higashi syndrome (oculocutaneous albinism with recurrent
infections; autosomal recessive)
(3) Hunter syndrome (MPS II)
(4) Sanfilippo-Good syndrome (MPS III)
Mosby. 1972.
PERIARTERITIS RETINALIS SEGMENTALIS

White or yellow plaques are arranged in segments encircling arteries like a cuff
and are localized to one or more arterial branches.
*1. Arteriosclerosis secondary to vein obstruction

2. Herpes zoster
3. Hypercholesterolemia
4. Lupus erythematosus (disseminated lupus erythematosus)
5. Metastatic uveitis
6. Periarteritis nodosa (necrotizing angiitis)
*7. Sarcoidosis syndrome
*9. Temporal arteritis (giant cell arteritis)
* 10. Tuberculous retinitis
11. Uveitis, idiopathic
Crouch ER, Goldberg MF. Retinal periarteritis secondary to syphilis. Arch Ophthalmol
1975; 93:384-387.
Rask AJ. Peri-arteritis retinalis segmentalis. Acta Ophthalmol 1969; 47:234-237.
FROSTED-BRANCH ANGITIS
In this condition, unusual thick sheathing surrounds all the retinal veins and less
often the arteries, making them look like frosted tree branches.
1. Herpes simplex viruses types 1 and 2

2. Acute lymphoblastic leukemia
3. Large cell lymphoma
4. Crohn disease
6. Acquired immune deficiency syndrome
Kim TS, et al. Retinal angiopathy resembling unilateral frosted branch angiitis in a
patient with relapsing acute lymphoblastic leukemia. Am J Ophthalmol 1994; 117, 6:806-
808.
Ridley ME, et al. Retinal manifestations of ocular lymphoma. J Ophthalmol 1992; 99,
7:1153-1161.
SHEATHING OF RETINAL VEINS

In this condition, white or gray envelopes are around veins; retinal or vitreous
hemorrhage and exudates may be present.
1. Disc only-developmental
2. Disc and retina-papillitis or papilledema
3. Peripheral sheathing
*A. Acute retinal necrosis
B. Amebiasis
C. Behçet disease (dermatostomatoophthalmic syndrome)
D. Brucellosis-rare, tortuosity and sheathing of veins, vitreous haze, retinal
hemorrhages
E. Candidiasis
F. Coccidioidomycosis
G. Eales disease (periphlebitis)
H. Diabetes mellitus
I. Filariasis-hemorrhages and exudates
J. Hypertension
K. Infectious mononucleosis-peripheral or central perivascular involvement,
venous engorgement and sheathing associated with retinal hemorrhages
L. Lupus erythematosus
M. Non-Hodgkin lymphoma
N. Onchocerciasis syndrome (river blindness)
O. Rickettsial infections-peripheral or central perivascular involvement, venous
engorgement and sheathing associated with retinal hemorrhages
*P. Sarcoidosis
Q. Septicemia and bacteremia-rare, venous engorgement, usually with multiple
hemorrhages and focal sheathing
R. Sickle cell disease
S. Syphilis (secondary) (acquired lues)
T. Tuberculin or bacille Calmette-Guerin (BCG) vaccination-rare. sectorial, or
generalized changes
U. Viral infections, including the following:
(2) Herpes simplex (likely responsible for acute retinal necrosis)
(3) Herpes zoster ophthalmicus
(4) Influenza
(5) Rift Valley fever
4. Peripheral sheathing without secondary retinopathy-multiple sclerosis
5. Wide and usually dense sheathing of dilated and tortuous veins, suggestive of
myelogenous leukemia
Brown S. et al. Intraocular lymphoma presenting as retinal vasculitis Surv Ophthalmol

1994;39:138-140.
George RK, et al. Primary retinal vasculitis. Opthalmology 1996:103:384-389.
Kohno T, et al. Ocular manifestations of adult T-cell leukemia/lymphoma.

Ophthalmology 1993; 100:1794-1799.
Roy FH. Ocular syndromes and systemic diseases. 3rd ed. Philadelphia Lippincott
ABSENT VENOUS PULSATIONS (SPONTANEOUS VENOUS PULSATIONS

ABSENT AT VENULES ON THE DISC)
1. Normal individuals
*2. Impending central vein occlusion (see p. 468)
*3. Papilledema (see p. 593-601)
Ballantyne AJ. Michaelson IC. Textbook of the fundus of the e) e. 3rd ed. Baltimore.
Newell FW. Ophthalmology: principles and practice. 7th ed. St. Louis: CV Mosby. 1991.
DILATED RETINAL VEINS
Normally, the arteriole-venule ratio is 2:3; with an increase in this ratio, the
retinal veins may be dilated.
1. Congenital
A. Congenital tortuosity of retinal vessels-rare, sometimes associated with
coarctation of aorta
B. Fabry disease (hereditary dystrophic lipidosis)
C. Hemangioma
D. Longfellow-Graether syndrome
E. Ocular fundi in newborns
F. Racemose (arteriovenous) aneurysm-rare, arteriovenous anastomoses localized
to sector of retina
*G. Retinopathy of prematurity with plus disease
H. von Hippel-Lindau disease (angiomatosis) - familial in 20% of cases, bilateral
in 50%
2. Trauma and inflammation
A. Anterior uveitis-dilatation of veins, often slight hyperemia of disc
B. Carotid-cavernous fistula-fracture of base of skull, progressive exophthalmos,
bruit
C. Cavernous sinus thrombosis-rare, proptosis and orbital edema
*D. Impending obstruction of the central retinal vein
E. Periphlebitis-sheathing of vessels
3. Cardiovascular disease-dilatation may be present but rarely dominates the fundus
picture
A. Arteriosclerosis
B. Involutionary sclerosis (later stages)
C. Secondary to defective arterial flow, such as in the following:
(1) Aortic arch syndrome (pulseless disease)
(2) Cardiac insufficiency
(3) Congenital heart disease
(4) Iatrogenic (lowering of blood pressure)
(5) Severe blood loss
*(6) Stenosis or occlusion of common carotid
*(7) Temporal arteritis
(8) Venous stasis (hypotensive retinopathy of Kearns and Hollenhorst)
D. Heritable thrombophilia and hypofibrinolysis
4; Respiratory disease-venous dilatation may occur with purplish hue of whole retina;
obstruction of venous return from the head, such as in the following:
A. Congenital septal defect (Fallot tetralogy)
B. Emphysema
C. Hamman-Rich syndrome (diffuse pulmonary fibrosis syndrome)
D. Heart failure of any type
E. Kartagener syndrome (sinusitis-bronchiectasis-situs inversus syndrome)
F. Mechanical compression of chest
G. Mediastinal tumor obstructing superior vena cava
5. Diseases of the central nervous system
*A. Carotid-cavernous fistula-fractured base of skull; rupture of berry aneurysm,
arteriosclerosis
B. Hemangioma of posterior fossa-rare, papilledema, often grossly dilated veins
C. Optic nerve lesion-rare, secondary to orbital space-occupying lesion
*D. Papilledema (see p. 593-601)
E. Retrolenticular syndrome (Dejerine-Roussy syndrome)
*F. Subarachnoid hemorrhage-head injury; subhyaloid hemorrhages near disc,
dilated veins, sometimes papilledema
6. Blood diseases
A. Aplastic anemia-hemorrhage is the most striking sign, often spreading around
the disc
B. Cryoglobulinemia-rare, may occur with multiple myeloma, veins dilated,
tortuous, and sometimes beaded
C. Gansslen syndrome (familial hemolytic icterus)
D. Lymphatic leukemia
E. Macrocytic anemia of all types-common, retinopathy absent unless hemoglobin
below 50%; pale fundus, superficial hemorrhages, cotton-wool spots
(2) Steatorrhea
(3) Celiac disease
(4) Carcinoma of stomach
F. Macroglobulinemia-rare; veins dilated tortuous and sometimes beaded,
hemorrhages and occasionally microaneurysms
G. Monocytic leukemia
H. Myelogenous leukemia
I. Multiple myeloma (Kahler disease)
J. Polycythemia rubra vera (primary; Vaquez disease)-common in males;
hemorrhages; papilledema may be marked and venous thrombosis may occur
K. Secondary polycythemia-common; hemorrhages, papilledema and venous
thrombosis may occur
L. Sickle cell disease-dilatation of peripheral veins with retinal, subhyaloid, and
vitreous hemorrhages
M. Thrombocytopenic purpura-retinal and subhyaloid hemorrhages near disc,
moderate venous dilatation
7. Acute febrile illnesses-rare, occasional dilatation of retinal veins with a few
hemorrhages and mild edema of disc
A. Infectious mononucleosis
B. Influenza
C. Rickettsial infections
D. Septicemia
8. Metabolic diseases
A. Cystic fibrosis syndrome (fibrocystic disease of pancreas)-venous congestion
often swelling of disc
B. Plasma lecithin
*C. Diabetic retinopathy-larger veins affected, often beaded
A. Polyarteritis nodosa-among other fundus lesions, dilated veins may occur
B. Sclerosis, progressive systemic (scleroderma)
C. Systemic lupus erythematosus-cotton-wool spots, occasional hemorrhages, and
moderate dilatation of veins
10. Toxic conditions, such as methyl alcohol ingestion
Glueck CJ, et al. Heritable thrombophilia and hypofibrinolysis-possible causes of retinal

vein occlusion. Arch Ophthalmol 1999; 117:43-49.
Newell FW. Ophthalmology: principles and practice, 7th ed. St. Louis: CV Mosby, 1991.
TORTUOSITY OF RETINAL VEINS AND HYPOPLASIA OF OPTIC NERVES
1. Endocrinopathy, especially pituitary deficiency

2. Alcohol abuse; fetal alcohol syndrome
3. Migraine disturbances
4. Agenesis of the corpus callosum associated with mutations
5. Preterm birth also associated with retinal artery/visceral tortuosity
Hellstrom A, et al. Optic nerve hypoplasia with isolated tortuosity of the retinal veins.
CENTRAL RETINAL VEIN OCCLUSION

This condition is characterized by massive hemorrhage into the posterior portion
of the eye and dilated retinal veins.
1. External compression of the vein

A. Atherosclerosis of central retinal artery
B. Connective tissue strand within the floor of the physiologic excavation
C. Multiple crossings of the same artery and vein or congenital venous loops or
twists in the retinal surface
D. Pseudotumor cerebri
2. Degenerative or inflammatory venous disease, causing detachment, proliferation, and
hydrops
A. Acquired immunodeficiency syndrome (AIDS; HIV retinopathy)
B. Arterial hypertension
C. Arteriovenous malformations of retina
D. Cardiac decompensation
E. Closed-head trauma
*F. Diabetes mellitus (Willis disease)
G. Ipsilateral internal carotid artery stenosis
H. Lyme disease
I. Optic disc drusen
J. Optic nerve inflammation
K. Sarcoidosis
L. Serpiginous choroiditis
M. Systemic granulomatous disease, particularly tuberculosis
3. Thrombosis from venous stagnation
A. Spasm of corresponding retinal arterioles
B. Blood dyscrasias
(1) Cryoglobulinemia
(2) Emphysema with secondary erythrocytosis
(3) Deficiencies in endemic pathway (factor V R506Q mutation)
(4) Homocystinemia
(5) Increased platelet aggregation
(6) Leukemias
(7) Lymphoma
*(8) Multiple myeloma
*(9) Polycythemia vera
(10) Sickle cell disease
C. Increased viscosity of the blood
(1) Cystic fibrosis of pancreas
(2) Following peritoneal dialysis
(3) Hyperproteinemia
*(4) Macroglobulinemia
(5) Use of tranexamic acid
D. Sudden reduction of systemic blood pressure because of cardiac
decompensation, surgical or traumatic shock, or therapy for arterial hypertension
*E. Glaucoma (preexisting)
F. Increased risk of thrombosis
(1) Hereditary
a. Antithrombin III deficiency
b. Protein C deficiency or protein S deficiency
c. Rare disorders of fibrinogen and fibrinolysis
(i) Certain dysfibrinogenemias
(ii) Abnormal plasminogen
(2) Acquired
a. Carcinoma
b. Hematologic proliferative disorders
(i) Acute promyelocytic leukemia
(ii) Myeloproliferative disorders (polycythemia, essential
thrombocythemia)
c. Behçet syndrome
d. Lupus anticoagulant
e. Nephrosis
f. Complications of therapy
(i) Oral contraceptives
(ii) Infusion of prothrombin complex concentrates
(iii) Heparin-induced thrombocytopenia
G. Carotid-cavernous sinus fistula
*H. Syphilis
I. With immunoglobulin G (IgG) lambda monoclonal gammopathy
J. Coil embolization of carotid-ophthalmic aneurysms
K. Oral contraceptive
Castillo B, et al. Retinal artery occlusion following coil embolization of carotid-

ophthalmic aneurysms. Arch Ophthalmol 2000; 118:851-852.
Enzenauer RW, et al. Central retinal vein occlusion in a patient with IgG lambda
monoclonal gammopathy. Arch Ophthalmol 1999; 117: 134-135.
Greiner K, et al. Retinal vascular occlusion and deficiencies in the protein C pathway. Am
J Opthalmol 1999:128: 69-74.
Hayreh SS, et al. Systemic diseases associated with various types of retinal vein
occlusion. Am J Ophthalmol 2001; 131:61-78.
Rath EZ, et al. Risk factors for retinal vein occlusions: a case-control study.
Sagripanti A, et al. Thrombin-antithrombin III complex in acute retinal vein occlusion.

Am J Ophthalmol 1999; 128, 1:124.
Wenzler EM, et al. Hyperhomocystinemia in retinal artery and retinal vein occlusion. Am
J Ophthalmol 1993; 115: 162-167.
Extracted Table Central Retinal Vein Occlusion
DILATED RETINAL VEINS AND RETINAL HEMORRHAGES
1. Carotid-cavernous fistula
*2. Cavernous sinus fistula syndrome (carotid artery syndrome)
3. Cavernous sinus thrombosis (hypophyseal-sphenoidal syndrome)
*4. Central retinal vein occlusion (see p. 468)
5. Cervical tuberculosis
6. Choroidal melanoma remote to the neovascularization
7. Congenital tortuosity and dilatation of the retinal vessels
8. Cryoglobulinemia
10. Intravitreal myiasis
11. Leukemia
12. Lymphomas
14. Multiple myeloma (myelomatosis)
16. Pappataci fever (phlebotomus fever)
17. Paraproteinemias and dysproteinemias
18. Polycythemia vera
19. Retinal arteritis
*21. Syphilis (acquired lues)
Kalina RE, Kaiser M. Familial retinal hemorrhages. Am J Ophthalmol 1972; 74:252-255.
RETINAL HEMORRHAGES
Retinal hemorrhages include bleeding that may be intraretinal or preretinal
hemorrhages into the vitreous or subretinal hemorrhages.
1. Congestion of the head and neck, such as in newborns, in hanging, or during choking
2. Trauma, including electrical injury, hypothermal injury, and child abuse
3. Vascular obstruction, such as cardiorespiratory obesity syndrome, cystic fibrosis
syndrome, negative acceleration syndrome, (hydrostatic pressure syndrome),
ophthalmoplegic migraine syndrome, papilledema (see p. 593-601), subarachnoid
hemorrhages, superior vena cava syndrome, Symonds syndrome (benign intracranial
hypertension), thrombocytopenia, thrombosis, and Wernicke syndrome (avitaminosis B1)
4. Inflammatory conditions, such as Criswick-Schepens syndrome (familial exudative
vitreoretinopathy), Loffler syndrome (eosinophilic pneumonitis), perivasculitis, and
subacute bacterial endocarditis
*5. Acute febrile and infectious illnesses, including amebiasis ankylostomiasis,
aspergillosis, bacterial endocarditis, coccidioidomycosis, cryptococcosis (torulosis),
cysticercosis, dengue fever, hydatid cyst (echinococcosis), hydrophobia (rabies),
infectious mononucleosis, influenza, Japanese River fever (typhus), lymphogranuloma
venereum (Nicolas-Favre disease), metastatic bacterial endophthalmitis, nematode
ophthalmia syndrome (toxocariasis), pertussis (whooping cough), Q fever, relapsing
fever, trichinellosis, Weil disease (leptospirosis), and yersiniosis
*6. Vascular disease, such as arteriosclerosis, atherosclerosis, arteriovenous fistula,
disseminated intravascular coagulation, hypertension, Paget syndrome (hypertensive
diencephalic syndrome), progressive systemic sclerosis, pulmonary insufficiency, the
retinopathies, particularly diabetic and hypertensive, and when the circulation through the
eye is diminished in hypotensive retinopathy, such as in carotid vascular insufficiency
syndrome or pulseless disease (Takayasu syndrome), suprarenal-sympathetic syndrome,
temporal arteritis syndrome (cranial arteritis syndrome), and in conditions of extreme
cachexia
7. Anemia that may be secondary to drugs, including the following:
acebutolol allobarbital amoxicillin
acenocoumarin allopurinol amphotericin B
acetaminophen alprazolam ampicillin
acetanilid aminopterin anisindione
acetazolamide aminosalicylic acid (?) antazoline
acetohexamide amithiozone antimony lithium
acetophenazine amitriptyline thiomalate
actinomycin C amobarbital antimony potassium
acyclovir amodiaquine tartrate
antimony sodium cefoxitin desipramine
tartrate cefsulodin dexbrompheniramine
antimony sodium ceftizoxime dexchlorpheniramine
thioglycollate ceftriaxone diazepam
antipyrine cefuroxime diazoxide
aprobarbital cephalexin dichlorphenamide
arninosalicylate (?) cephaloglycin dicloxacillin
atenolol cephaloridine dicumarol
auranofin cephalothin diethazine
aurothioglucose cephapirin diltiazem
aurothioglycanide cephradine dimercaprol
azatadine chlorambucil dimethindene
azathioprine chloramphenicol dimethyl sulfoxide
barbital chlordiazepoxide diphenadione
BCG vaccine chloroquine diphenhydramine
bendroflumethiazide chlorothiazide diphenylhydantoin
benzathine penicillin G chlorpheniramine diphenylpyraline
benzthiazide chlorpromazine diphtheria and tetanus
bishydroxycoumarin chlorpropamide toxoids and pertussis
bleomycin chlorprothixene vaccine (adsorbed)
brompheniramine chlortetracycline doxorubicin
busulfan chlorthalidone doxycycline
butabarbital cholecalciferol doxylamine
butalbital cimetidine dromostanolone
butallylonal cisplatin droperidol
butaperazine clemastine enalapril
butethal clindamycin ergocalciferol
cactinomycin clofibrate erythromycin
calcitriol clonazepam ethacrynic acid
captopril clorazepate ethopropazine
carbamazepine cloxacillin ethosuximide
carbenicillin colchicine ethotoin
carbimazole cyclobarbital ethoxzolamide
carbinoxamine cyclopentobarbital ethyl biscoumacetate
carisoprodol cyclophosphamide fenfluramine
carmustine cycloserine fenoprofen
carphenazine cyclosporine flecainide
cefaclor cyclothiazide floxuridine
cefadroxil cyproheptadine fluorouracil
cefamandole cytarabine fluoxymesterone
cefazolin dacarbazine fluphenazine
cefonicid dactinomycin flurazepam
cefoperazone dapsone furosemide
ceforanide daunorubicin gentamicin
cefotaxime deferoxamine glutethimide
cefotetan demeclocycline glyburide
gold Au 198 mefenamic acid nortriptyline
gold sodium thiomalate melphalan oral contraceptives
gold sodium thiosulfate mephenytoin orphenadrine
griseofulvin mephobarbital oxacillin
guanethidine meprobamate oxazepam
halazepam mercaptopurine oxyphenbutazone
haloperidol mesoridazine oxytetracycline
heparin methacycline paramethadione
heptabarbital methaqualone penicillamine
hetacillin metharbital pentobarbital
hexethal methazolamide perazine
hexobarbital methdilazine periciazine
hydrabamine methicillin perphenazine
penicillin V methimazole phenacetin
hydrabamine methitural phenelzine
phenoxymethyl methohexital phenformin
penicillin methotrexate phenindione
hydralazine methotrimeprazine pheniramine
hydrochlorothiazide methsuximide phenobarbital
hydroflumethiazide methyclothiazide phenprocoumon
hydroxychloroquine methyldopa phensuximide
hydroxyurea methylene blue phenylbutazone
ibuprofen methylphenidate phenytoin
imipramine methylthiouracil pindolol
indapamide methyprylon piperacetazine
indomethacin metolazone piperazine
influenza virus vaccine metoprolol pipobroman
interferon metrizamide poliovirus vaccine
isocarboxazid metronidazole polythiazide
isoniazid mexiletine potassium penicillin G
labetalol mianserin potassium penicillin V
levodopa midazolam potassium phenethicillin
lincomycin minocycline prazepam
lithium carbonate mitomycin primidone
lomustine moxalactam probarbital
lorazepam mumps virus vaccine procaine penicillin G
loxapine (live) procarbazine
maprotiline nadolol prochlorperazine
measles and rubella nafcillin promazine
virus vaccine (live) nalidixic acid promethazine
measles virus vaccine naproxen propiomazine
(live) nialamide propylthiouracil
measles, mumps and nifedipine protriptyline
rubella virus vaccine nitrazepam pyrilamine
(live) nitrofurantoin pyrimethamine
mechlorethamine nitroglycerin quinacrine
quinethazone testolactone triethylenemelamine
quinidine testosterone trifluoperazine
quinine tetracycline trifluperidol
ranitidine thiabendazole triflupromazine
rifampin thiamylal trimeprazine
rubella and mumps virus thiethylperazine trimethadione
vaccine (live) thioguanine tripelennamine
rubella virus vaccine thiopental triprolidine
(live) thiopropazate uracil mustard
secobarbital thioproperazine urethan
semustine thioridazine vancomycin
sodium thiotepa verapamil
antimonylgluconate thiothixene vidarabine
stibocaptate tocainide vinbarbital
stibogluconate tolazamide vinblastine
stibophen tolazoline vincristine
streptomycin tolbutamide vitamin A
sulfonamides tranylcypromine vitamin D
suramin trazodone vitamin D2
talbutal triazolam vitamin D3
temazepam trichlormethiazide warfarin
8. Vascularized neoplasms, including hereditary hemorrhagic telangiectasia (Rendu-

Osler-Weber disease), and periocular and ocular metastatic tumors
acetylcholine cobalt (?) isosorbide
acid bismuth sodium cocaine ketoprofen
tartrate cortisone lincomycin
adrenal cortex injection deserpidine mannitol
aldosterone desoxycorticosterone medrysone
allopurinol (?) dexamethasone meprednisone
alseroxylon dibucaine methaqualone
aspirin dyclonine methylphenidate
benoxinate epinephrine methylprednisolone
betamethasone ethambutol mithramycin
bismuth carbonate fludrocortisone mitotane
bismuth oxychloride fluorometholone oxyphenbutazone
bismuth salicylate fluorouracil paramethasone
bismuth sodium tartrate fluprednisolone penicillamine
bismuth sodium glycerin phenacaine
thioglycollate heparin phenylbutazone
bismuth sodium hexachlorophene piperocaine
triglycollamate hydrocortisone plicamycin
bismuth subcarbonate indomethacin pralidoxime
bismuth subsalicylate iodide and iodine prednisolone
butacaine solution compounds prednisone
proparacaine sulfadimethoxine sulfathiazole
radioactive iodides sulfamerazine sulfisoxazole
rauwolfia serpentina sulfameter sulindac
rescinnamine sulfamethazine syrosingopine
reserpine sulfamethizole tamoxifen
sodium chloride sulfamethoxazole tetracaine
sodium salicylate sulfamethoxypyridazine triamcinolone
sulfacetamide sulfanilamide trichloroethylene
sulfachlorpyridazine sulfaphenazole urea
sulfacytine sulfapyridine urokinase (?)
sulfadiazine sulfasalazine vitamin A
10. Hematopoietic system, such as the anemias, Bing-Neel syndrome (association of

macroglobulinemia and central nervous system symptoms), Fanconi syndrome (amino
diabetes), Gansslen syndrome (familial hemolytic icterus), Henoch-Schönlein purpura,
Herrick syndrome (sickle cell disease), Jacobsen-Brodwell syndrome, leukemias,
hemophilia, polycythemia, purpuras, oculootoororenoerythropoietic disease, Plummer-
Vinson syndrome (sideropenic dysphagia syndrome), Reimann syndrome (hyperviscosity
syndrome), Waldenstrom syndrome (macroglobulinemia syndrome), Wiskott-Aldrich
syndrome (purpura), also following blood transfusion with incompatibility of blood
groups
11. Acosta syndrome (mountain climber syndrome)
12. Amyloidosis
16. Epidural steroid injection, gas myelography, or epiduroscopy
17. Following labor induced by oxytocin or dinoprostone in newborns
18. Following use of YAG laser
20. HIV -related retinopathy
21. Hodgkin disease
22. Juvenile diabetes-dwarfism-obesity syndrome
23. Macular degeneration, age related (exudative type)
24. Morning-glory syndrome (hereditary central glial anomaly of the optic disc)
25. Mycosis fungoides syndrome (Sézary syndrome)
26. Neuroblastoma
27. Optic nerve drusen (see p. 559-560)
29. Plasma lecithin (cholesterol acyltransferase deficiency)
31. Polymyositis dermatomyositis (Wagner-Unverricht syndrome)
33. Purtscher retinopathy
34. Radiation retinopathy
36. Schamberg disease (self-limiting cutaneous vasculitis)
37. Terson syndrome
Amirikia A, et al. Acute bilateral visual loss associated with retinal hemorrhages
following epiduroscopy. Arch Ophthalmol 2000; 118:287-288.
Kivlin JD, et al. Shaken baby syndrome. Ophthalmology 2000; 107:1246-1254.
Obana A, et al. Retinal and sublyaloid hemorrhage as a complication of laser iridectomy

for primary angle-closure glaucoma. Arch Ophthalmol 2000; 118:1449-1451.
Roy FH. Ocular syndromes and systemic diseases, 300 ed. Philadelphia: Lippincott
Yu T, et al. Epidemiology and biostatistics. Arch Opthalmol 1998; 116:83-89.
LARGE HEMORRHAGES IN THE FUNDUS OF AN INFANT OR YOUNG

CHILD (SUGGESTIVE OF INCREASED INTRACRANIAL PRESSURE AND
PARALYSIS OF CRANIAL NERVES)
1. Hygroma
*2. Shaken-baby syndrome
3. Subarachnoid hemorrhage
Drack AV. et al. Unilateral retinal hemorrhages in documented cases of child abuse. Am J
Ophthalmol 1999; 128.3: 340-344.
Lambert SR. et al. Optic nerve sheath and retinal hemorrhages associated with the shaken
baby syndrome. Arch Ophthalmol 1986; 104:1509-1512.
Nover A. The ocular fundus: methods of examination and typical findings, 4th ed.
RETINOVITREAL HEMORRHAGE IN A YOUNG ADULT
1. Incontinentia pigmenti (Bloch-Sulzberger syndrome)

2. Congenital x-linked (juvenile) retinoschisis
*4. Sickle cell anemia
5. Trauma
6. von Hippel-Lindau syndrome
Duke-Elder S. System of ophthalmology. Vol. X. St. Louis: CV Mosby, 1967.
Morse PH. Vitreoretinal disease-a manual for diagnosis and treatment, 2nd ed. St. Louis:
CV Mosby. 1989.
RETINAL HEMORRHAGE WITH CENTRAL WHITE SPOT (ROTH SPOT)
1. Collagen disease
2. Cyanosis retinae-carcinoma of the lung
4. Following heart surgery
5. Following uncomplicated pediatric cataract extraction
*6. Hematopoietic system
A. Anemias
B. Leukemia
C. Multiple myeloma (Kahler disease)
7. Intracranial hemorrhage (infants)
8. Septic retinitis
*A. Candida albicans infection
B. Kala azar
C. Phlebitis
D. Rheumatic mitral and aortic valvulitis
E. Rocky mountain spotted fever
*F. Subacute bacterial endocarditis
G. Syphilitic aortitis
H. Viral pneumonia
9. Vascular disease
Extracted Table Retinal hemorrhage with Central White Spot
Mets MB, Del Monte M. Hemorrhagic retinopathy following uncomplicated pediatric

cataract extraction. Arch Ophthalmol 1986; 104:975.
Roy FR. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott
MICROANEURYSMS OF RETINA (PUNCTATE RED SPOTS SCATTERED

OVER REGION OF POSTERIOR POLE)
1. Aging
2. Aplastic anemia-punctate hemorrhage
3. Associated with cotton-wool spots (see p. 491)
4. Bonnet-Dechaume-Blanc syndrome (cerebroretinal arteriovenous aneurysm syndrome)
5. Choroiditis
6. Chronic uveitis
*12. Hypertension
13. Hypotensive retinopathy, such as pulseless disease (aortic arch syndrome)
14. Kahler disease (myelomatosis)
15. Leukemias-punctate hemorrhages
16. Loa loa infection
18. Mauriac syndrome (juvenile diabetes-dwarfism-obesity syndrome)
19. Ocular ischemic syndrome (carotid occlusive disease)
20. Osler hemorrhagic telangiectasia (hereditary hemorrhagic telangiectasis)
21. Pelizaeus-Merzbacher syndrome (aplasia axialis extracorticalis congenita)
*23. Retinoblastoma
24. Sickle cell hemoglobin C disease
25. Skin divers
26. Subacute bacterial endocarditis
27. Venous occlusion-occlusion of central retinal vein or one of its branches (see p. 468)
Polkinghorne PJ, et al. Ocular fundus lesions in divers. Lancet 1988; Dec: 1381-1383.
Sanders RJ, et al. Foveal avascular zone diameter and sickle cell disease. Arch
Ophthalmol 1991; 109:812-816.
Yu T, et al. Epidemiology and biostatistics. Arch Opthalmol 16:83-89.
Extracted Table Microaneurysms of Retina
RETINAL ARTERIOVENOUS SHUNT AT THE ARTERIOVENOUS CROSSING
2. Leber's miliary aneurysm
3. Retinal vein occlusion
4. Takayasu disease
Tanaka T, et al. Retinal arteriovenous shunt at the arteriovenous crossing. Ophthalmology

1998; 105:1251-1258.
Tanaka T, Shimizu K. Retinal arteriovenous shunts in Takayasu disease. Ophthalmology

1987; 94:1380-1388.
MACROANEURYSMS OF RETINAL ARTERIES
These macroaneurysms are found within the first three orders of bifurcation of
arterioles; they are frequently associated with localized hemorrhage and exudation.
1. Congenital
*2. Generalized arteriosclerosis
*3. Hypertension
4: Idiopathic
5: Following open heart surgery
Kuhn F. Retinal emboli after open heart surgery. Arch Ophthalmol 1989; 107:317.
Robertson DM. Macroaneurysm of the retinal arteries. Trans Am Acad Ophthalmol

Otolaryngol 1973; 77:55-67.
Schatz H. Essential fluorescein angiography: a compendium of classical cases. San

Anselmo, CA: Pacific Press, 1982.
RETINAL NEOVASCULARIZATION (GROWTH OF ABNORMAL NEW

BLOOD VESSELS INTO THE VITREOUS)
1. Anemia
*3. Central retinal vein occlusion (see p. 468)
7. Hypertension (malignant and essential)
8. Leukemia
9. Lupus erythematosus
11. Retinal detachment with hemorrhage
14. Trauma
16. Werlhof disease (hemophilia and thrombocytopenic purpura)
L'Esperance FA, James WA. Diabetic retinopathy: clinical evaluation and management,
2nd ed. St. Louis: CV Mosby, 1982.
PREDISPOSITION TO RHEGMATOGENOUS RETINAL DETACHMENT

1. Aphakia (see p. 405)
2. Branch retinal vein occlusion
3. High myopia
4. Chorioretinitis
5. Peripheral retinal degeneration
A. Vitreous base excavation
*B. Retinal hole
C. Retinoschisis
D. Cystic retinal tuft
E. Zonular traction tuft
F. Meridional folds
G. Partial-thickness retinal tear
*H. Full-thickness retinal tear
*I. Lattice degeneration
J. Vitreous base avulsion
6. Trauma-blunt and perforating, including operation for strabismus and block excision of
epithelial implantation cysts and tumors of the anterior uvea
7. Angiomatosis retinae
Gonzales CA, et al. Bilateral rhegmatogenous retinal detachments with unilateral vitreous
base avulsion as the presenting signs of child abuse. Am J Ophthalmol 1999; 127, 4:475-
477.
Ikuno Y, et al. Tractional retinal detachment after branch retinal vein occlusion.
Ophthalmology 1998; 105:417 -423.
Jonas JB, et al. Rhegmatogenous retinal detachment after block excision of epithelial
implantation cysts and tumors of the anterior uvea. Ophthalmology 1999; 106:1942-1946.
RETINAL DETACHMENT (LOCATION AND MORPHOLOGIC

CLASSIFICATION)
1. Equator
A. Myopic type-equatorial horseshoe tear
B. Equatorial type associated with lattice degeneration
2. Ora serrata
A. Aphakic, with multiple small breaks often in nasal periphery
B. Dialysis in young, lower temporal quadrant, often bilateral
C. Giant dialysis, often bilateral
3. Posterior pole
A. Macular breaks, rare
B. Other breaks at posterior pole, from cellular proliferation in inner retinal
surface
Benson WE. Retinal detachment: diagnosis and management, 2nd ed. Philadelphia: JB
Lippincott, 1988.
Bhagat N, et al. Exudative retinal detachment in relapsing polychondritis. Ophthalmology

2000; 108:1156-1159.
Folk JC, Burton TC. Bilateral phakic retinal detachment. Ophthalmology 1982; 89:815.
SYNDROMES AND DISEASES ASSOCIATED WITH RETINAL

DETACHMENT
1. Exudative
A. Systemic disease
(1) Abdominal typhus
(2) Aspergillosis
(3) Atopic dermatitis
(4) Blood diseases
a. Dysproteinemias
b. Leukemia
c. Sickle cell disease
(5) Boutonneuse fever (rickettsia)
(6) Candidiasis
(7) Coenurosis
(9) Cryptococcosis
(10) Cysticercosis
(11) Disseminated intravascular coagulation
(12) Extreme venous congestion, such as occurs during choking
(13) Goldsheider syndrome (epidermolysis bullosa)
(14) Goodpasture syndrome (chronic relapsing pulmonary hemosiderosis)
(15) Grönblad-Strandberg syndrome (systemic elastodystrophy)
(16) Histiocytosis X (Hand-Schüller-Christian syndrome)
(17) Homocystinuria syndrome
(18) Hurler syndrome (MPS I-H)
(19) Hydatid cyst
(20) Hypertension-grade IV
(21) Krause syndrome (congenital encephalo-ophthalmic dysplasia)
*(22) Lupus erythematosus
(23) Lymphoma
(25) Reese syndrome (D trisomy)
(26) Regional enteritis
(28) Renal disease, including chronic glomerulonephritis or uremia
(30) Rheumatic fever
(31) Rift Valley fever
(32) Sturge-Weber syndrome (meningocutaneous syndrome)
(33) Syphilis
(34) Temporal arteritis syndrome (cranial arteritis syndrome)
(35) Toxemia of pregnancy
(36) Vogt-Koyanagi-Harada syndrome
B. Ocular disease
(2) Choroidal or retinal tumor
a. Hemangioma
b. Melanoma
c. Metastasis-including that from breast, lung, and stomach
d. Retinoblastoma
(3) Colobomas of the optic nerve
(4) Dominant myopia and retinal detachment
(5) Familial exudative vitreoretinopathy
(6) Harada disease and Vogt-Koyanagi syndrome
(7) Lymphoid hyperplasia of the uveal tract
(8) Morning-glory syndrome (hereditary central glial anomaly of the optic
disk)
(9) Nanophthalmos
(10) Norrie disease (atrophia oculi congenita)-x-linked
(11) Optic nerve pit
(12) Postinflammation of the orbit or sinuses or cyclitis
(13) Retina, congenital nonattachment and falciform folds-autosomal
recessive
(14) Schwartz syndrome (glaucoma associated with retinal detachment)
(15) Scleritis (especially posterior scleritis)
(16) Sympathetic ophthalmia
(17) Toxocara infection
(18) Uveal effusion syndrome
C. Associated with retinal or choroidal vascular disease
(1) Coats disease (retinal telangiectasia)
a. In juvenile
b. In adult
(3) Central serous choroidopathy
(4) Detached pigment epithelium
(5) Eales disease (periphlebitis)
(6) Excessive panphotocoagulation
(7) Exudative age-related macular degeneration
(8) Hollenhorst syndrome (chorioretinal infarction syndrome)
(9) Incontinentia pigmenti
(10) Osteoporosis-pseudoglioma syndrome
(11) Post irradiation
(12) Scleral buckling
(13) Subpigment epithelium hemorrhage
(14) von Hippel Lindau disease (retinocerebral angiomatosis)
aceclidine echothiophate oxygen
adrenal cortex injection fludrocortisone oxyphenbutazone
aldosterone fluorometholone penicillamine
beclomethasone fluprednisolone phenylbutazone
betamethasone ganciclovir physostigmine (?)
carbachol hydrocortisone pilocarpine
chymotrypsin (?) isoflurophate (?) prednisolone
cortisone medrysone prednisone
demecarium (?) meperidine sane paramethasone
dexamethasone methyl prednisolone triamcinolone
diisopropyl methylphenidate
flurophosphate (DFP) neostigmine (?)
2. Traction
*A. Pull of adherent and degenerated vitreous
B. Organized vitreous band
(1) After vitreous hemorrhage
a. Spontaneous
b. Traumatic
(2) Hypertensive retinopathy
(3) Posthemorrhagic proliferative retinopathy
(4) Sickle cell retinopathy
C. Postneovascularization of vitreous
*(1) Diabetic retinopathy, proliferative
(2) Eales disease (periphlebitis)
(3) Ehlers-Danlos syndrome (fibrodysplasia elastica-generalisata)
(4) Fibrinoid syndrome
*(5) Retinopathy of prematurity
(6) Severe uveitis
D. Congenital deformities, such as retinal dysplasia, coloboma, persistence of
fetal vascular system, and pit of optic nerve
E. 18Q syndrome
F. Penetrating injury
*G. Proliferative vitreoretinopathy
H. Puckering syndrome
I. Retinal disinsertion syndrome
J. Retinopathy of prematurity
K. Warburg syndrome
3. Rhegmatogenous
A. Accommodation spasm, including strong miotics
B. Alport syndrome (neuropathy and deafness)
C. Apert syndrome (acrocephalosyndactylism syndrome)
D. Equatorial or anterior choroiditis
E. FOAR syndrome
F. Following YAG laser capsulotomy
G. Hereditary ocular vitreoretinal degeneration and skeletal abnormality (cleft
palate)
H. Juxtapapillary microholes
I. Knobloch syndrome (retinal detachment and occipital encephalocele)-
autosomal recessive
J. Marchesani syndrome
K. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)
L. Marshall (D) syndrome
M. Meckel syndrome
N. Myopia, including staphyloma-autosomal dominant or recessive
O. Retinal degeneration at periphery
(1) Presenile or myopic type
(2) Lattice and paving-stone types-autosomal dominant
P. Retinal detachment-autosomal dominant or x-linked
Q. Retinal vein occlusion
R. Retinoschisis-adult or juvenile
S. Smith-Magenis syndrome
T. Spondyloepiphyseal dysplasia, congenital
U. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)
V. Trauma
(1) Direct injury-perforating wound and foreign body
(2) Indirect injury including block excision of epithelial implantation cysts
and tumors of the anterior uvea.
(3) Post cataract operation
a. Sunset syndrome
b. Vitreous tug syndrome
(4) Battered-baby syndrome (Silverman syndrome)
*W. Viral retinitis
X. Vitreous degeneration
Y. Wagner syndrome (hyaloideoretinal degeneration)
Alio JL, et al. Retinal detachment as a potential hazard in surgical correction of severe
myopia with phakic anterior chamber lenses. Am J Ophthalmol 1993; 115:145-148.
Hunyor AP, et al. Ocular-central nervous system lymphoma mimicking posterior scleritis
with exudative retinal detachment. Ophthalmology 2000; 107:1955-1959.
Ikuno Y, et al. Retinal detachment after branch retina vein occlusion. Ophthalmology
1997; 104:27-32.
Jonas JB, et al. Rhegmatogenous retinal detachment after block excision of epithelial
implantation cysts and tumors of the anterior uvea. Ophthalmology 1999; 106:1942-1946.
Ranta P, Kivela T. Retinal detachment in pseudophakic eyes with and without Nd:YAG
laser posterior capsulotomy. Ophthalmology 1998; 105:2127-2133.
RETINAL FOLDS
1. Proliferative retinal folds-inner layer outstrips outer layer

2. Traction folds
A. Associated with remnants of hyaloid artery
B. Secondary to vitreous traction
*C. Retinopathy of prematurity (cicatricial form)
3. Falciform retinal fold (congenital retinal septum)
A. Familial exudative vitreoretinopathy
B. Isolated
C. Trisomy syndrome
D. Warburg syndrome
4. Chronic uveitis
5. Parasite
6. Occult intraocular foreign body
*7. Shaken baby syndrome
8. Terson syndrome
Keithahn MA, et al. Retinal folds in Terson syndrome. Ophthalmology 1993; 100:1187-
1190.
Larrison WI, et al. Posterior retinal folds following vitreoretinal surgery. Arch
Ophthalmol 1993; 111:621-625.
COTTON-WOOL SPOTS
These spots are soft exudates (fluffy, white, focal infarcts in the nerve fiber layer).
1. Acute pancreatitis
2. Amniotic fluid embolization
3. Anemic conditions
A. Cirrhosis of the liver
B. Following cardiac surgery
C. Gastric ulcer syndrome
D. Hypotensive retinopathy
E. Ligation of the carotid artery
F. Severe primary and secondary anemias
G. Severe systemic blood loss
4. Blood disease
A. Aplastic anemia
B. Dysproteinemia
C. Leukemia
D. Multiple myeloma (myelomatosis)
E. Pernicious anemia (vitamin B12 deficiency)
F. Waldenström syndrome (macroglobulinemia syndrome)
5. Carbon monoxide poisoning
6. Carcinomatous cachexia
A. Dermatomyositis (polymyositis dermatomyositis)
B. Diffuse scleroderma
C. Disseminated lupus erythematosus (systemic lupus erythematosus)
D. Polyarteritis nodosa (necrotizing angiitis)
E. Rheumatoid arthritis with scleromalacia perforans or polymyalgia rheumatic a
*8. Diabetic retinopathy
9. Hodgkin disease
10. Infective conditions
*A. HIV
B. Pneumonia
C. Rheumatic fever
D. Rift Valley fever
E. Rocky mountain spotted fever (spotted fever)
F. Roth septic retinitis
G. Subacute bacterial endocarditis
11. Microemboli following cardiac operation
12. Primary amyloidosis (idiopathic amyloidosis)
13. Primary open-angle glaucoma
14. Protein C and protein S deficiency
15. Purtscher retinopathy (fat embolism syndrome)
16. Renal disease
17. Serum disease
18. Suprarenal-sympathetic syndrome (pheochromocytoma syndrome)
19. Takayasu syndrome (aortic arch syndrome)
20. Toxemic retinopathy of pregnancy
*21. Untreated malignant hypertension
Brezin A, et al. Cotton-wool spots and AIDS-related complex. Int Ophthalmol 1990;
14:37-41.
Mausour AM, et al. Cotton-wool spots in acquired immunodeficiency syndrome

compared with diabetes mellitus, systemic hypertension, and central retinal vein
occlusion. Arch Ophthalmol 1988; 106:1074-1077.
Extracted Table Cotton-wool spots
HARD EXUDATES (YELLOWISH WHITE DISCRETE MASSES DEEP IN THE

RETINA)
1. Circinate retinopathy
*4. Exudative age-related macular degeneration
*5. Hypertensive disease
6. Radiation induced
7. Retinal arterial macroaneurysm
Berman DH, Friedman EA. Partial absorption of hard exudates in patients with diabetic
end-stage renal disease and severe anemia after treatment with erythropoietin. Retina
1994; 14:1-5.
Haik BG, et al. Radiation and chemotherapy of parameningeal rhabdomyosarcoma

involving the orbit. Ophthalmology 1986; 93:1001-1009.
Williams &Wilkins, 2002.
Extracted Table Hard Exudates
RETINAL EXUDATE AND HEMORRHAGE
1. Capillary telangiectasis of retina (Reese)

5. Multiple retinal aneurysms (Leber syndrome)
6. Racemose hemangioma of the retina
7. von Hippel-Lindau, with absence of visible angioma (retinocerebral angiomatosis)
Ballantyne AJ, Michaelson IC. Textbook of the fundus of the eye, 3rd ed. Baltimore:
Michelson JB, et al. Ocular reticulum cell sarcoma. Arch Ophthalmol 1981; 99:1409.
RETINITIS OR PSEUDORETINITIS PIGMENTOSA

Pigment may be bone corpuscular dots or heaped-up masses; salt and pepper
fundus
A. Abetalipoproteinemia (Bassen-Kornzweig syndrome)
B. Alström disease (cataract and retinitis pigmentosa)
C. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
D. Dialinas-Amalric syndrome (deaf-mutism-retinal degeneration syndrome)
E. Hallgren syndrome (retinitis pigmentosa-deafness-ataxia syndrome)
F. Hypotrichosis, syndactyly and retinitis pigmentosa-autosomal recessive
G. Hunter syndrome (MPS II)
H. Hurler syndrome (MPS I)
I. Infantile phytanic acid storage disease
J. Jeune syndrome
*K. Kearns-Sayre syndrome (ophthalmoplegic retinal degeneration syndrome)
L. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-
M. Metaphyseal chondrodysplasia with retinitis pigmentosa-autosomal recessive
N. Microcephaly with chorioretinopathy
O. Multiple sulfatase deficiency
P. Muscular atrophy, ataxia, retinitis pigmentosa, diabetes mellitus-autosomal
dominant
Q. Olivopontocerebellar atrophy, type III
R. NARP syndrome
S. Pallidal degeneration, progressive with retinitis pigmentosa-autosomal
recessive
*T. Refsum syndrome (phytanic acid storage disease)
U. Retinitis pigmentosa alone (usually autosomal recessive but may be autosomal
dominant or sex linked)
V. Retinitis pigmentosa associated with myopia, keratoconus, or glaucoma
W. Retinitis pigmentosa, congenital deafness-sex linked
X. Retinitis pigmentosa inversa (predominant-pigmentation around the disc and
macula) and deafness-autosomal recessive
Y. Retinitis pigmentosa, nerve deafness, mental retardation, and hypogonadism -
autosomal recessive
Z. Retinitis pigmentosa, PPRPE type (with preserved para-arteriole retinal
pigment epithelium) - autosomal recessive
AA. Retinitis pigmentosa, spastic quadriplegia, and mental retardation-autosomal
recessive
BB. Rud syndrome
CC. Sanfilippo disease (MPS III)
DD. Scheie disease (MPS IS)
EE. Spielmeyer-Vogt syndrome (cerebroretinal degeneration)
FF. Usher syndrome (hereditary retinitis pigmentosa-deafness syndrome)
2. Senile changes-degenerative pigmentation
3. Vascular lesion, such as occlusion of arteriole
4. Inflammatory
A. Behçet disease (oculobuccogenital syndrome)
B. Chickenpox virus
C. Cytomegalic inclusion disease
D. Dawson disease (inclusion-body encephalitis)
E. Fetal varicella effects
F. Focal dermal hypoplasia (Goltz syndrome)
G. Harada disease (Vogt-Koyanagi-Harada syndrome)
H. Hypomelanosis of Ito
I. Influenza virus
J. Nematode endophthalmitis (visceral larva migrans syndrome)
K. Onchocerciasis (river blindness)
L. Polyarteritis nodosa (Kussmaul disease)
M. Rubella (German measles)
N. Rubeola (measles)
*O. Syphilis
*P. Toxoplasmosis
Q. Typhoid fever (enteric fever)
R. Vaccinia
5. Toxic
A. Accidental intraocular injection of depot corticosteroids
B. Chloroquine and atabrine
C. Diaminodiphenoxyalkanes-possible drug for treatment of schistosomiasis
D. Indomethacin
E. Phenothiazine
(1) Chlorpromazine
(2) Thioridazine (Mellaril)
F. Pregl solution (Septojod, formerly used for treatment of puerperal sepsis)
G. Quinine
H. Sparsomycin
6. Acute lymphocytic leukemia
8. Alport syndrome
9. Bardet-Biedl syndrome
10. Battens disease
11. Cryogenic "pigmentary fallout" - following use of cryosurgery for retinal detachment
13. External ophthalmoplegias
*14. Gardner syndrome (congenital hypertrophy of the retinal pigment epithelium and
familial intestinal polyposis)
15. Hagberg-Santavuori syndrome (neuronal ceroid lipofuscinosis)
17. Hereditary ataxias (Friedrich and Marie)
19. Lens dislocated into vitreous
20. MERRF syndrome
21. Mucolipidoses IV (ML IV)
23. Neuronal ceroid lipofuscinosis
A. Infantile form
B. Late infantile form (Jansky-Bielschowsky)
C. Adult form (Kufs syndrome)
24. Pelizaeus-Merzbacher syndrome (aplasia axialis extracorticalis congenita)
25. Pellagra (ariboflavinosis)
26. Progressive cone-rod degeneration
27. Renal disorders, including familial juvenile nephronophthisis (medullary cystic
disease)
28. Rud syndrome (hypophyseal deficiency)
29. Sjögren-Larsson syndrome (oligophrenia-ichthyosis-spastic diplegia syndrome)
30. Tapetal-like reflex syndrome
31. Trauma, including blunt, penetrating, obstetric, and radiotherapy, Frenkel syndrome
(ocular contusion syndrome)
33. Zellweger syndrome and pseudo-Zellweger syndrome
Bateman JB, Philippart M. Ocular features of the Hagberg-Santavuori syndrome. Am J

Ophthalmol 1986; 102: 262-271.
Bloome MA, Garcia CA. A manual of retinal and choroidal dystrophies. East Norwalk,
CT: Appleton-Century-Crofts, 1982.
Carr RE, Noble KG. Retinitis pigmentosa. Ophthalmology 1981; 88:199.
Extracted Table Retinal bone corpuscular dots
LESIONS CONFUSED WITH RETINOBLASTOMA
1. Anomalous optic disc

2. Anteriorly dislocated lens with secondary glaucoma
4. Coloboma of choroid and optic disc (see p. 555-556)
5. Congenital corneal opacity
6. Congenital rubella syndrome (Gregg syndrome)
7. Cysts in a remnant of the hyaloid artery
8. Developmental retinal cyst
9. Glioma of the retina
10. Hematoma under retinal pigment epithelium
11. High myopia with advanced chorioretinal degeneration
12. Juvenile (x-linked) retinoschisis
13. Juvenile xanthogranuloma (nevoxanthoendothelioma)
* 14. Larval granulomatosis (T. canis)
15. Medullation of nerve fiber layer
16. Metastatic endophthalmitis
17. Norrie disease (atrophia oculi congenital
18. Oligodendroglioma of the retina
19. Organization of intraocular hemorrhage
*20. Persistent hyperplastic primary vitreous
21. Retinal detachment due to choroidal or vitreous hemorrhage
22. Retinal dysplasia (massive retinal fibrosis)
24. Retrolental membrane associated with Bloch-Sulzberger syndrome (incontinentia
pigmenti)
25. Rhegmatogenous and falciform retinal detachment
26. Secondary glaucoma
27. Sex-linked microphthalmia
28. Tapetoretinal degeneration
29. Trisomy 13 (Patau syndrome)
*30. Toxoplasmosis (ocular toxoplasmosis)
31. Traumatic chorioretinitis
*32. Tumors other than retinoblastoma
33. Uveitis in secondary retinal detachment
34. "White-with-pressure" sign
Howard GM. Erroneous clinical diagnoses of retinoblastoma and uveal melanoma. Trans
Am Acad Ophthalmol Otolaryngol 1969; 73:199-203.
Nicholson DR. Green WR, eds. Pediatric ocular tumors. New York: Masson. 1981.
Extracted Table Single dark fundus lesion
SINGLE WHITE LESION OF RETINA
*1. Amelanotic melanoma

2. Astrocytoma of tuberous sclerosis
3. Degeneration of retinal pigment epithelium
4. Diktyoma
5. Glioma of optic nerve
6. Hamartomas of the optic disc of retinitis pigmentosa
7. Metastatic or direct extension of a tumor
8. Neurofibroma of von Recklinghausen syndrome
*9. Retinoblastoma
* 10. T. canis
Nicholson DH, Green WR, eds. Pediatric ocular tumors. New York: Masson, 1981.
Robertson DM. Hamartomas of the optic disc with retinitis pigmentosa. Am J Ophthalmol
1972; 74:526-531.
PALE FUNDUS LESIONS
1. Generalized pallor
A. Albinism-photophobia; defective vision; absence of pigment in iris, retina.
And choroid
B. Chediak-Higashi syndrome (oculocutaneous albinism with recurrent
infections)
C. Waardenburg syndrome (embryonic fixation syndrome)
D. Choroideremia-rare; night blindness; contraction of visual fields; degeneration
of pigment epithelium in periphery with exposure of choroidal vessels
E. Myopia-thinning of retina and choroidal crescent at disc
F. Retinal ischemia
(1) Occlusion of retinal arteries (see p. 457)
(2) Spasm of retinal arteries - angiospasm: quinine, lead poisoning,
migraine, or Raynaud disease
(3) Anemia
*G. Vascular retinopathies-hypertension, edema. hemorrhages, swelling of disc
H. Leukemia
I. The lipidoses
(1) Congenital, rare
(2) Infantile (Tay-Sachs disease)
(3) Late infantile - (Jansky-Bielschowsky syndrome) - 2 to 4 years of age
(4) Juvenile - (Spielmeyer-Vogt syndrome) - 5 to 8 years of age; optic
atrophy
(5) Adult - (Kufs disease) - 15 to 25 years of age; eyes may be normal or
show some pigmented macular changes
J. Gaucher disease (glucocerebroside storage disease)
K. Hereditary dystrophic lipidosis (Fabry disease)
L. Hyperlipemia
(1) Diabetes-rare, yellowish retinal and choroidal vessels
(2) Essential hyperlipemic xanthomatosis-rare, yellowish retinal and
choroidal vessels
M. Oguchi disease
2. Localized pale areas
A. Medullated nerve fibers (see p. 507)
*B. Retinopathy of prematurity
C. Localized retinal edema
(1) Inflammation
(2) Trauma
(3) Vascular lesions
D. Retinal detachment and schisis (see p. 487)
*E. Retinoblastoma
F. Coats disease (retinal telangiectasia)
G. Coloboma (see p. 450-451)
H. Normal fundus features-pale streaks mark site of ciliary nerves
I. Atrophic areas-diathermy, light coagulation, or cryosurgery
J. Scattered retinal exudates
(1) Preretinal-severe posterior uveitis; discrete white spots, often most
marked along vessels adjacent to a patch of choroiditis
(2) Retinal
a. Purtscher compression syndrome-cotton-wool spots
b. Fat emboli
c. Hemangiomatosis-yellow exudates
*d. Hypertensive retinopathy-cotton-wool and hard exudates
e. Toxemia of pregnancy
f. Hypotensive retinopathy
g. Pulseless disease (Takayasu syndrome)
h. Arterial occlusion
i. Blood loss-cotton-wool spots
j. Anemia (all types)
k. Leukemia
l. Purpura
m. Macroglobulinemia (Waldenström syndrome)
n. Hodgkin disease-soft exudates
*o. Diabetes-cotton-wool and hard exudates
p. Hypercholesterolemia-lipid deposits
q. Systemic lupus erythematosus (disseminated lupus
erythematosus)
r. Dermatomyositis-cotton-wool spots
s. Polyarteritis nodosa (Kussmaul disease)
t. Scleroderma (progressive systemic sclerosis)
u. Vitamin A deficiency-small white spots along course of retinal
vessels
v. Retinal capillariosis-yellowish white spots in substance of retina
w. Leber congenital retinal aplasia-bilateral blindness, multiple
white specks
x. Female carrier of retinitis pigmentosa-brilliant silvery reflex
with shining yellow spots deep to retinal vessels
3. Dystrophic conditions
A. Gyrate atrophy - rare, irregular atrophic areas with visual defects and night
blindness
B. Choroidal sclerosis-rare, diffuse peripapillary or central choroidal atrophy with
larger choroidal vessels prominent
C. Infarction or occlusion of ciliary arteries-rare, embolism (air, fat), injury,
atrophic area with prominent choroidal vessels
D. Pseudoinflammatory macular dystrophy-rare, fourth to sixth decades, central
edema, hemorrhage and exudate, bilateral and symmetric
E. Helicoid peripapillary chorioretinal atrophy-rare, congenital and adult forms,
star-shaped atrophic areas radiating from disc
F. Retinitis punctata albescens-rare, onset in second and third decades, multiple
discrete whitish dots which may appear crystalline, night blindness and field
defects in progressive type
G. Fundus flavimaculatus-rare, onset in second and third decades, yellow flecks
deep in the retina
H. Geographic choroiditis-rare, map-like pigmentary disturbance at posterior pole
or more widespread over posterior fundus
I. Doyne honeycomb dystrophy-rare; middle age and older; drusen at posterior
pole, with pigmentary or cystoid macular changes
J. Progressive bifocal chorioretinal atrophy-atrophy temporal to disc, extending
later; night blindness in late stage
Bloome MA, Garcia CA. Manual of retinal and choroidal dystrophies. East Norwalk,
CT: Appleton-Century-Crofts. 1981.
Perkins ES, Dobree JR. The differential diagnosis of fundus conditions. St. Louis: CV
Mosby, 1972.
MEDULLATED NERVE FIBERS

In this condition, an opaque white patch is usually adjacent to and may cover the
disc; it is localized to one sector of the disc and peripapillary or arcuate with a peripheral,
feathered edge.
*1. Isolated finding

2. Autosomal-recessive or -dominant inheritance
3. Associated with the following:
A. Aplasia of macula
B. Coloboma of optic nerve or choroid (see p. 555-556)
C. Conus of disc
D. Cranial dysostosis (oxycephaly, dolichocephaly, brachycephaly, and
craniofacial dysostosis)
E. Hyaloid remnants
F. Macular colobomas (see p. 450)
G. Myopia
*H. Neurofibromatosis
Ballantyne AJ, Michaelson IC. Textbook of the fundus of the eye. 3rd ed. Baltimore:
PIGMENTED FUNDUS LESIONS

1. Diffuse pigmentation
A. Negroid fundus-accentuation of fundus pigmentation
B. Melanosis bulbi-rare, pigmentation of external eye and fundus
C. Nevus of Ota
D. Waardenburg syndrome (embryonic fixation)
2. Single pigmented lesions
A. Flat lesions
(1) Benign melanoma-bluish, gray, or black lesion
(2) Pigmented scar-patch of dense pigment, usually atrophic area in center
(3) Fuchs dark spot-dark spot in macular region
*(4) Macular degeneration (exudate, age-related)
B. Raised lesions
(1) Simple detachment (see p. 487)
a. Macular, such as in central serous retinopathy
b. Associated with uveitis, such as that associated with Vogt-Koya-
nagi-Harada syndrome
c. Hemorrhagic macrocyst
*(2) Malignant melanoma-raised pigmented lesion with secondary
detachment, abnormal vessels
(3) Choroidal hemorrhage-trauma, spontaneous in patients with vascular
disease, high myopia
*(4) Exudative macular lesion - common, old age, subretinal exudate
(5) Hemangioma of choroid - rare, raised grayish tumor near disc.
Secondary detachment later
*(6) Metastatic tumor - flat tumor with little pigment, primary in breast, or
lung
(7) Chorioretinitis
(8) Foreign body
(9) Coats disease (retinal telangiectasia)
3. Multiple pigmented lesions
A. Scattered focal lesions
*(1) Congenital melanosis - cat's-paw patches of pigment in one sector of
fundus (may be part of Gardner syndrome)
(2) Postinflammatory - flat pigment with areas of atrophy
(3) Hypertensive retinopathy-hypertensive vascular changes with scattered
pigmentation
(4) Siegrist streaks-rare, chain of pigment spots along sclerosed choroidal
vessel
(5) Paravenous retinochoroidal atrophy - paravenous pigmentation with
chorioretinal atrophy
(7) Chorioretinal scars from cryosurgery
B. Widely disseminated pigmentary changes
(1) Genetic conditions
a. Typical retinitis pigmentosa-attenuation of retinal vessels, optic
atrophy (myopia, posterior polar cataract, keratoconus)
b. Atypical retinitis pigmentosa-rare, little or no pigment, pigment
in clumps
c. Retinitis pigmentosa syndromes
(i) Cockayne syndrome (dwarfism with retinal atrophy and
deafness)
(ii) Hallgren syndrome (retinitis pigmentosa-deafness-
ataxia syndrome)
(iii) Kearns syndrome (ophthalmoplegic retinal
degeneration syndrome)
(iv) Laurence-Moon-Biedl syndrome (retinitis pigmentosa
polydactylyadiposogenital syndrome)
(v) Leber congenital retinal aplasia syndrome
(vi) Lignac-Fanconi syndrome (cystinosis syndrome)
(vii) Myotonic dystrophy syndrome (dystrophia myotonica
syndrome)
(viii) Pelizaeus-Merzbacher syndrome (aplasia axialis
extracorticalis congenital)
(2) Infectious conditions-secondary retinitis pigmentosa
*a. Syphilis (congenital)-pepper-and-salt pigmentation, interstitial
keratitis
b. Syphilitic neuroretinitis-rare, retinitis pigmentosa
c. Rubella-cataract, secondary retinitis pigmentosa
(nonprogressive)
d. Vaccinia-rare, retinitis pigmentosa, history of vaccination
(3) Metabolic Disturbances
a. Refsum syndrome (phytanic acid storage disease)
b. Bassen-Kornzweig syndrome (familial hypolipoproteinemia)
(4) Toxic conditions, such as chloroquine, phenothiazine derivatives;
usually central pigmentation; cornea and lens change
4. Ciliary body and choroid
A. Tumors
(1) Hemangioma
*(3) Metastatic carcinoma, such as that from the lungs, breast, testis,
kidney, prostate gland, bladder
(4) Nevus
(5) Neurilemmoma
(6) Neurofibroma
B. Detachment -serous or hemorrhagic
*C. Lymphoma and leukemias
D. Peripheral giant cysts
5. Vitreous body
A. Hemorrhage
B. Abscess
6. Staphyloma of sclera
Mosby, 1972.
Zinn K. Marmor M, eds. The retinal pigment epithelium. Cambridge. MA: Harvard
CHOLESTEROL EMBOLI OF RETINA (HOLLENHORST PLAQUES)

Bright-yellow plaques are often observed at bifurcation of arterioles, indicative of
generalized atherosclerosis, and should signal the ophthalmologist to measure retinal
artery pressures and refer the patient for general medical evaluation.
1. Abdominal aortic aneurysms

*2. Aortic stenosis
3. Arteriography showing occlusions in one or more cervical arteries
4. Atrial fibrillation
5. Bleeding duodenal or gastric ulcer
*6. Bruits in one or both carotid arteries
*7. Calcification of internal carotids (Doppler ultrasonography)
8. Congestive heart failure
9. Coronary heart disease with myocardial infarct or angina
11. New or old strokes or transient attacks of cerebral ischemia
12. Peripheral atherosclerosis obliterans, popliteal or femoral aneurysms
13. Renal artery occlusions
*14. Retinal arterial occlusions
15. Torsion and calcification of aorta (roentgenogram)
16. Vocal cord paralysis (aortic arch aneurysm)
Pfaffenbach DD, Hollenhorst RW. Morbidity and survivorship of patients with embolic
cholesterol crystals in the ocular fundus. Am J Ophthalmol 1973; 75:66-72.
Wylie EJ, Ehrenfeld WK. Extracranial occlusive cerebrovascular disease: diagnosis and
management. Philadelphia: WB Saunders, 1970.
RETINAL MICROEMBOLI
1. Platelet fibrin-mural or "tail" thrombus in carotid occlusion

*2. Cholesterol-lipid-eroding atheroma in carotid bifurcation
3. Calcific or fibrinoid
A. Calcific valvular disease dislodged spontaneously following cardiac
catheterization, or angiography, or prolapse of mitral valve
B. Rheumatic heart disease
C. Myocardial disease
D. Septic emboli
4. Foreign bodies
A. Silicone or cloth particles covered cardiac valves
B. Talc or cornstarch emboli from drug addicts
C. Mercury
D. Secondary to retrobulbar or intranasal methyl prednisolone acetate
5. Tumors
A. Cardiac myxomas
B. Metastatic tumors including malignant melanomas and breast carcinomas
6. Fat emboli from fracture of the long bones
7. Air emboli from crushing injuries of the chest
Fraunfelder FT, Roy FH. Current ocular therapy. 5th ed. Philadelphia: WB Saunders.
2000.
Williams IM, et al. Brain and retinal microemboli during cardiac surgery. Ann Neurol
1991; 30:736-737.
LIPEMIA RETINALIS (ARTERIOLES AND VENULES SIMILAR IN COLOR

AND APPEARING ORANGE-YELLOW TO WHITE)
1. Primary hyperlipoproteinemia
A. Type I-familial fat-induced hyperlipoproteinemia (hyperchylomicronemia)
B. Type III-familial hyperbetalipoproteinemia and hyperprebetalipoproteinemia
(carbohydrate- induced hyperlipemia)
C. Type IV -familial hyperprebetalipoproteinemia (carbohydrate-induced
hyperlipemia)
D. Type V-familial hyperchylomicronemia with hyperprebetalipoproteinemia
(mixed hyperlipemia)
*2. Diabetes mellitus with hyperlipemia
3. Secondary hyperlipoproteinemia
A. Biliary obstruction
B. Chronic pancreatitis
C. Chronic renal failure
D. Coats disease in adults (retinal telangiectasia)
E. Glycogen storage disease
F. Hypergammaglobulinemia
G. Hypothyroidism (cretinism)
H. Idiopathic hypercalcemia
I. Insulin-deficient diabetes mellitus (Willis disease)
*J. Malignant neoplasms
K. Nephrotic syndrome (lipoid nephrosis)
L. Progressive lipodystrophy
Martinez KR, et al. Lipemia retinalis. Arch Ophthalmol 1992; 110.1171.
Spaeth GL. Ocular manifestations of the lipidoses. In Tasman W. ed. Retinal diseases in
children. New York: Harper & Row, 1971.
HEMORRHAGIC OR SEROUS EXUDATES BENEATH PIGMENT
EPITHELIUM
1. Angioid streaks (see p. 526-529)

2. Best macular degeneration (vitelliruptive macular dystrophy)
4. Doyne honeycomb macular degeneration
*5. Histoplasmosis (histoplasmosis choroiditis)
*6. Macular drusen in age-related macular degeneration
7. Myopia
8. Solid neoplasms
9. Trauma
Gitter KA, et al. Traumatic hemorrhagic detachment of retinal pigment epithelium. Arch
Ophthalmol 1968; 79:729- 732.
Pager CK, et al. Malattia leventinese presenting with subretinal neovascular membrane
and hemorrhage. Am J Ophthalmol 2001; 131:517-518.
RETINAL VASCULAR TUMORS AND ANGIOMATOSIS RETINAE

SYNDROMES
1. Associated with pheochromocytoma

2. Blue rubber bleb nevus syndrome (Bean syndrome)
3. Bonnet-Dechaune-Blanc syndrome (neuroretinal angiomatosis syndrome)
4. Cavernous retinal hemangioma - intraretinal angiomas
6. Gorlin syndrome
7. Racemose angioma - with arteriovenous anomalies of central nervous system
(Wyburn-Mason syndrome)
8. Retinal telangiectasis (Leber military aneurysms) - telangiectasia retinae of Reese
9. Sturge- Weber syndrome (meningocutaneous syndrome)
*10. von Hippel-Lindau syndrome (retinocerebral angiomatosis retinae)
Crompton JL, Taylor D. Ocular lesion in the blue rubber nevus syndrome.
De Potter P, et al. Combined hamartoma of the retina and retinal pigment epithelium in
Gorlin syndrome. Arch Ophthalmol 2000; 118: 1004-1006.
Geeraets WJ. Ocular syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.
Extracted Table Retinal vascular tumors

Extracted Traumatic retinopathies
RETINAL "SEA-FANS"
These are vasoproliferative lesions with a characteristic fan-shaped appearance,
also called a "parachute" lesion.
1. Aortic arch syndrome (pulseless disease)

2. Carotid-cavernous fistula (carotid artery syndrome)
*3. Central and branch retinal vein occlusion (see p. 468)
4. Chronic myelocytic leukemia
7. Facioscapulohumeral muscular dystrophy (FSH [facio scapulo numeral] syndrome)
8. Incontinentia pigmenti I (Block-Sulzberger syndrome)
9. Familial exudative vitreoretinopathy (Criswick-Schepens syndrome)
10. Hemoglobin C trait
11. Leukemia
12. Long-standing retinal detachment
13. Lupus erythematosus
14. Macroglobulinemia
16. Polycythemia vera (erythremia)
18. Sarcoidosis syndrome
* 19. Sickle cell disease
20. Talc and cornstarch emboli
21. Uveitis, including pars planitis
Gurwin EB, et al. Retinal telangiectasis in facioscapulohumeral muscular dystrophy with

deafness. Arch Ophthalmol 1985; 103: 1695-1707.
Jampol LM, Goldberg MH. Peripheral proliferative retinopathies. Surv Ophthalmol 1980;
25:1-14.
Rodgers R, et al. Ocular involvement in congenital leukemia. Am J Ophthalmol 1986;

101:730-732.
RETINAL VESSELS DISPLACED TEMPORALLY

1. Familial vitreoretinopathy
2. Hamartomas
3. Inflammation
4. Myopia with lattice-like retinal degeneration
*6. Sickle cell disease (drepanocytic anemia)
7. Trauma
Nover A. The ocular fundus: methods of examination and typical findings. 4th ed.
RETINAL VESSELS DISPLACED NASALLY
1. Axial myopia
*2. Glaucoma
3. Inflammation
4. Trauma
Ballantyne AJ, Michelson IC. Textbook of the fundus of the eye. 3rd ed. Baltimore:
Nover A. The ocular fundus: methods of examination and typical findings. 4th ed.
PERIPHERAL FUNDUS LESIONS
1. Pale raised lesions

A. Vitreous opacities-white fluffy or discrete opacities, associated with pars
planitis or sarcoid uveitis
B. Retinopathy of prematurity-retinal edema and dense white lesions with
neovascularization
C. Toxocariasis (nematode ophthalmia syndrome)-vitreous opacities with
peripheral granuloma
D. Leprosy (Hansen disease)-peripheral exudates with anterior uveal involvement
E. Vitreoretinal dystrophies-bands in vitreous with retinoschisis or retinal
detachment
F. Angiomatosis-retinal tumor with enlarged, feeding vessels
*G. Retinoblastoma-raised, creamy-white, fluffy lesion without inflammatory
signs
2. Flat lesions
A. Coloboma-pale area with pigmented edge in region of fetal cleft
B. Chorioretinitis
(1) Disseminated, congenital syphilis-pepper-and-salt or larger confluent
lesions
(2) Toxoplasmosis-pigmented scars of old lesions
(3) Cytomegalic inclusion disease-localized chorioretinitis or general
peripheral infiltration
*(4) Histoplasmosis-peripheral punched-out lesions with or without
pigmentation
C. Peripheral degenerations
(1) Senile changes-of eyes older than years of age, depigmented areas with
pigmented margins (cobblestone degeneration).
(2) Secondary pigmentary degeneration-peripheral pigmentary changes
similar to senile type or to retinitis pigmentosa
(3) Cystoid degeneration-multiple cystic spaces and thin areas in
peripheral retina
*(4) Lattice degeneration-lace work of white lines with depigmented and
pigmented patches
(5) Cystinosis (cystine storage aminoaciduria dwarfism syndrome)-
granular rings of pigment in periphery of fundus, similar to cobblestone
degeneration
D. Equatorial linear pigment disturbance
(1) Ophthalmomyiasis internal
(2) Histoplasmosis syndrome
E. Retinitis
*(1) Acute retinal necrosis
*(2) Cytomegalovirus retinitis
3. Dark raised lesions
A. Choroidal detachment (see p. 532-535)
(1) Spontaneous-slowly progressive detachment, no inflammatory signs
*(2) Postoperative-intraocular operation; particularly for cataract and
glaucoma; shallow anterior chamber; leaking wound
(3) Exudative
a. Inflammatory-shallow anterior chamber, myopia, and peripheral
detachment
b. Vascular-nephritis, hypertension, toxemia of pregnancy,
polyarteritis nodosa, leukemia
*c. Tumors-intraocular tumors; tumors of orbit and lacrimal gland
d. Traumatic-contusion injuries, perforating wounds, hypotony,
anterior chamber may be shallow or deep if perforation occurs
posteriorly
B. Exudative retinal detachment (see p. 487)
*(1) Secondary to general disease with retinopathy-hypertension, toxemia
of pregnancy, leukemia, dysproteinemia, polyarteritis nodosa, rickettsial
arteritis, venous congestion, talc and cornstarch emboli
(2) Secondary to local disease of the eye-inflammatory signs with
exudative detachment. Harada disease, sympathetic ophthalmitis, scleritis,
tenonitis, choroidal tumor, and ophthalmomyiasis
C. Simple detachment-myopia in two thirds of patients, trauma, may follow
cataract extraction or discission for congenital cataract
D. Cysts
(1) Ciliary body-larger cysts usually push iris forward; rarely, cyst extends
backward to be seen ophthalmoscopically
(2) Pars plana-may enlarge and appear as a multilocular reddish-brown
cyst
E. Scleral indentation-retinal detachment operation
F. Neoplasms of ciliary body
(1) Benign epithelioma - brown spot to 2 mm in diameter on surface of
ciliary body
*(2) Other tumors - diktyoma, leiomyoma, reticuloses, neurofibroma,
malignant melanoma, rare, usually present as a mass protruding through
the root of iris; may cause glaucoma; dark bulge seen
ophthalmoscopically; lens changes adjacent to tumor
G. Neoplasms of choroid
(1) Congenital melanosis-cat's-paw patches of pigment in one sector of
fundus
(2) Choroidal nevus-flat, bluish gray or black lesion
*(3) Malignant melanoma-raised, pigmented lesion with secondary
detachment
(4) Secondary metastatic-rare, primary lesion in breast, lung, and so on
4. Vascular lesions
A. Periphlebitis (Eales disease) common; young adults; sheathing of peripheral
veins; hemorrhages in new vessels and later retinal detachment (see p. 487)
*B. Perivasculitis secondary to uveitis - perivascular infiltration, particularly in
pars planitis, sarcoidosis, Behçet disease, and toxoplasmosis
C. Systemic diseases
(1) Rickettsia-engorgement of veins, retinal edema, hemorrhages, and
exudates
(2) Multiple sclerosis (disseminated sclerosis) - sheathing of veins (see p.
468)
(3) Polyarteritis nodosa (necrotizing angiitis) - hemorrhages, exudates, and
serous detachment of retina (see p. 488)
(4) Tuberculin or BCG inoculation-rare, sheathing of peripheral veins with
hemorrhages
*(5) Sickle cell retinopathy (Herrick syndrome) - dilatation of peripheral
veins, hemorrhages, and connective tissue sheets in periphery; new vessel
formation
Mason GI. Bilateral ophthalmomyiasis interna. Am J Ophthalmol 1981; 91:65-70.
Perkins ES. Dobree JH. The differential diagnosis of fundus conditions. St. Louis: CV
Mosby. 1972.
Tasman W, Shields JA. Disorders of the peripheral fundus. New York: Harper & Row.
1980.
RETINAL DISEASE ASSOCIATED WITH CORNEAL PROBLEMS
1. Abdominal typhus (enteric fever)-corneal ulcer, retinal detachment, central retinal

artery emboli
2. Acanthamoeba-keratitis, pannus, corneal ring abscess, retinal perivasculitis
3. African eyeworm disease-keratitis, central retinal artery occlusion, macular
hemorrhages
4. Amyloidosis - amyloid corneal deposits, corneal dystrophy, retinal hemorrhages
opacification, malformed retina with retina pseudotumors
6. Angioedema (hives)-central serous retinopathy, corneal edema
*7. Anterior segment ischemia syndrome-corneal edema midperiphery retinal
hemorrhages
8. Apert syndrome (acrodysplasia)-exposure keratitis, retinal detachment
9. Arteriovenous fistula - bullous keratopathy, retinal hemorrhages
10. Aspergillosis-corneal ulcer, keratitis, retinal hemorrhages, retinal detachment
11. Atopic dermatitis-keratoconus and retinal detachment
12. Avitaminosis C-retinal hemorrhages, keratitis, corneal ulcer
13. Bacillus cereus-ring abscess of cornea, necrosis of retina
14. Bang disease (brucellosis)-keratitis, chorioretinitis, macular edema
15. Behçet syndrome (dermatostomata-ophthalmic syndrome)-keratitis, posterior corneal
abscess, retinal vascular changes
16. Bietti disease (Bietti marginal crystalline dystrophy)-marginal corneal dystrophy,
retinitis punctate albescens
17. Candidiasis-keratitis, corneal ulcer, retinal atrophy, retinal detachment
*18. Carotid artery syndrome-corneal ulcer, loss of corneal sensation, retinal edema,
engorgement of retinal veins
19. Chickenpox (varicella)-corneal ulcer, corneal opacity, retinitis, hemorrhagic
retinopathy
20. Chloroquine-corneal epithelial pigmentation, macular lesions
21. Chronic granulomatous disease of childhood-keratitis, destructive chorioretinal
lesions
22. Cockayne syndrome (dwarfism with retinal atrophy and deafness)-pigmentary
degeneration, band keratopathy, corneal dystrophy
23. Crohn disease (granulomatous ileocolitis)-marginal corneal ulcers, keratitis, macular
edema, macular hemorrhages
24. Cryoglobulinemia-deep corneal opacities, venous stasis
25. Cystinosis (aminoaciduria)-crystals in cornea and pigment in retina
26. Dengue fever-keratitis, corneal ulcer, retinal hemorrhages
27. Diffuse keratoses syndrome-corneal nodular thickening in the stroma worse in fall,
retinal phlebitis
28. Diphtheria-keratitis, corneal ulcer, central artery occlusion
29. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)-keratitis,
keratoconjunctivitis sicca, corneal ulcer, central retinal vein occlusion, retinal detachment
30. Ehlers-Danlos syndrome (cutis hyperelastica) - keratoconus, and retinitis pigmentosa
31. Electrical injury-corneal perforation, retinal edema, retinal hemorrhages, pigmentary
degeneration, retinal holes, dilatation of retinal veins
32. Fabry disease (diffuse angiokeratosis)-whorl-like changes in cornea, central retinal
artery occlusion, tortuosity of retinal vessels
33. Goldscheider syndrome (epidermolysis bullosa)-bullous keratitis with opacities,
retinal detachment
34. Gronblad-Strandberg syndrome (systemic elastodystrophy) - angioid streaks of the
retina, macular hemorrhages, retinal detachment, keratoconus
35. Hamman-Rich syndrome (alveolar capillary block syndrome)-keratomalacia ischemic
retinopathy, cystic macular changes
36. Heerfordt syndrome (uveoparotid fever)-band keratopathy, retinal vasculitis
37. Hennebert syndrome (luetic otitic nystagmus syndrome) - interstitial keratitis,
disseminated syphilitic chorioretinitis
38. Histiocytosis X (Hand-Schüller-Christian syndrome)-retinal hemorrhage, retinal
detachment, bullous keratopathy, corneal ulcer, pannus
39. Hodgkin disease-keratitis, retinal hemorrhages
40. Hollenhorst syndrome (chorioretinal infarction syndrome)-hazy cornea, serous retinal
detachment, pigmentary retinopathy
41. Hunter syndrome (MPS Il)-splitting or absence of peripheral Bowman membrane,
stromal haze, pigmentary retinal degeneration, narrowed retinal vessels
42. Hurler-Scheie syndrome (MPS IH-S)-corneal clouding, pigmentary retinopathy
43. Hurler syndrome (gargoylism)-diffuse corneal haziness, retinal pigmentary changes,
megalocornea, retinal detachment
44. Hydatid cyst (echinococcosis)-keratitis, abscess of cornea, retinal detachment, retinal
hemorrhages
45. Hyperlipoproteinemia-arcus juvenilis, lipemia retinalis, xanthomata of retina
46. Hyperparathyroidism-band keratopathy, vascular engorgement of retina
47. Hypovitaminosis A-keratomalacia with perforation, corneal opacity, retinal
degeneration
48. Idiopathic hypercalcemia (blue-diaper syndrome)-band keratopathy, optic atrophy,
papilledema
49. Indomethacin-corneal deposits, reduced retinal sensitivity
50. Influenza-keratitis, retinal hemorrhage
51. Japanese River fever (typhus)-keratitis, retinal hemorrhages
52. Juvenile rheumatoid arthritis (Still disease)-band keratopathy, macular edema
53. Kahler disease (multiple myeloma)-crystalline deposits of cornea, central retinal
artery occlusion, retinal microaneurysms
54. Kussmaul disease (periarteritis nodosa)-retinal detachment, pseudoretinitis
pigmentosa, corneal ulcer
55. Leber tapetoretinal dystrophy syndrome (retinal aplasia)-keratoconus, salt-and-pepper
or "bone corpuscle" pigmentation, yellowish brown or gray macular lesions
56. Lubarsch-Pick syndrome (primary amyloidosis)-amyloid corneal deposits, retinal
hemorrhages
*57. Lymphogranuloma venereum disease (Nicolas-Favre disease)-keratitis, pannus,
corneal ulcer, keratoconus, tortuosity of retinal vessels, retinal hemorrhages
58. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)-keratoconus,
retinitis pigmentosa
59. Meckel syndrome (dysencephalia splanchnocystic syndrome) - sclerocornea,
microcornea, retinal dysplasia
60. Meningococcemia-keratitis, retinal endophlebitis
61. Mikulicz-Radeski syndrome (dacryosialoadenopathy)-keratoconjunctivitis, retinal
candlewax spots
62. ML IV (mucolipidosis IV)-corneal clouding, corneal opacities, retinal atrophy
63. Morbilli (measles-rubeola)-keratitis, corneal ulcer, pigmentary retinopathy, central
retinal artery occlusion
64. Mucormycosis (phycomycosis)-corneal ulcer, striate keratopathy, retinitis, central
retinal artery thrombosis
65. Mycosis fungoides syndrome (malignant cutaneous reticulosis syndrome)-
keratoconjunctivitis, retinal edema, retinal hemorrhage
66. Myotonic dystrophy syndrome-corneal epithelial dystrophy, loss of corneal
sensitivity, tapetoretinal degeneration, macular red spot, macular degeneration,
chorioretinitis
*67. Neurofibromatosis (von Recklinghausen syndrome)-nodular swelling nerves,
hamartoma of retina
68. Norrie disease (atrophia oculi congenita)-malformation of sensory cells of retina,
corneal nebulae
69. Oculodental syndrome (Peters syndrome)-corneoscleral staphyloma, megalocornea,
corneal marginal opacities, macular pigmentation
70. Onchocerciasis syndrome-punctate keratitis, sclerosing keratitis, chorioretinitis,
retinal degeneration
71. Paget syndrome (osteitis deformans)-corneal ring opacities, retinal hemorrhages,
pigmentary retinopathy, macular changes resembling Kuhnt-Junius degeneration
72. Phenothiazine-epithelial and endothelial pigment, retinal pigmentation
73. Pierre Robin syndrome (micrognathia-glossoptosis)-retinal disinsertion,
megalocornea
74. Plasma lecithin (cholesterol acyltransferase deficiency)-corneal stromal opacities,
retinal hemorrhages
75. Porphyria cutanea tarda - keratitis, retinal hemorrhages, cotton-wool spots, macular
edema
76. Postvaccinial ocular syndrome-corneal vesicles, and marginal ulcers, chorioretinitis,
central serous retinopathy, central retinal vein thrombosis
77. Progressive systemic sclerosis-marginal corneal ulcers with cicatrization, cotton-wool
spots, retinal hemorrhages
78. Radiation-corneal ulcer, punctate keratitis, keratoconjuctivitis sicca, retinal
hemorrhage, macular degeneration, macular holes with vascularization
*79. Refsum syndrome (phytanic acid oxidase deficiency)-band keratopathy, retinitis
pigmentosa
80. Relapsing fever-interstitial keratitis, retinal hemorrhage
81. Relapsing polychondritis-corneal ulcer, retinal detachment, retinal artery thrombosis,
82. Renal failure-cotton-wool spots, band keratopathy
83. Rendu-Osler syndrome (hereditary hemorrhagic telangiectasis) - intermittent
filamentary keratitis, small retinal angiomas, retinal hemorrhages
84. Retinal disinsertion syndrome-bilateral keratoconus, retinal detachment
*85. Retinoblastoma-corneal neovascularization, retinal tumor
86. Rothmund syndrome (telangiectasia-pigmentation cataract syndrome)-corneal lesions,
retinal hyperpigmentation
87. Rubella syndrome (Gregg syndrome)-microcornea, pigmentary retinal changes
88. Sabin-Feldman syndrome-microcornea, chorioretinitis or atrophic degenerative
chorioretinal changes
89. Sanfillipo-Good syndrome (mucopolysaccharidosis III)-slight narrowing of retinal
vessels, acid mucopolysaccharide deposits in cornea.
*90. Schaumann syndrome (sarcoidosis syndrome)-mutton fat keratitic precipitates,
keratitis sicca, band-shaped keratitis, inflammatory retinal exudates
91. Scheie syndrome (MPS I-S)-diffuse to marked corneal clouding, tapetoretinal
degeneration
92. Schwartz syndrome (glaucoma associated with retinal detachment)-retinal
detachment, microcornea
93. Shy-Gonatas syndrome (orthostatic hypotension syndrome)-keratopathy, corneal
ulcer, lattice-like white opacities in the area of Bowman membrane, retinal pigmentary
degeneration
94. Smallpox-keratitis, congenital corneal clouding, chorioretinitis
95. Stannus cerebellar syndrome (riboflavin deficiency)-corneal vascularization,
superficial diffuse keratitis, corneal opacities, brownish retinal patches
chorioretinal degeneration, total retinal detachment
97. Sturge-Weber syndrome (neurooculocutaneous angiomatosis)-retinal detachment,
increased corneal diameter with cloudiness
*98. Syphilis (acquired lues)-keratitis, retinal hemorrhages, retinal proliferation
*99. Temporal arteritis syndrome (Hutchinson-Horton-Magath-Brown syndrome)-retinal
detachments, narrowing of retinal vessels, central retinal artery occlusion, corneal
hypesthesia
100. Trisomy 13 (Patau syndrome)-malformed cornea, retinal dysplasia
*101. Tuberculosis-keratitis, pannus, corneal ulcer, retinitis
102. Ullrich syndrome (dyscraniopygophalangy)-cloudy cornea, corneal ulcers,
chorioretinal coloboma
103. Ultraviolet radiation-photokeratitis, band keratopathy, herpes simplex keratitis,
recurrent corneal erosions, retinal degeneration
104. Vaccinia-keratitis, pannus, corneal perforation, central serous retinopathy,
pseudoretinitis pigmentosa
105. van Bogaert-Scherer-Epstein (primary hyperlipidemia)-arcus juvenilis of the cornea,
lipid keratopathy, retinopathy with yellowish deposits
106. Vitreous tug syndrome-vitreous strands attached to corneal wound or scar,
circumscribed retinal edema, posterior retinal detachment
107. von Gierke disease (glycogen storage disease type I)-corneal clouding, discrete
nonelevated, yellow flecks in macula
108. Waardenburg syndrome (embryonic fixation syndrome)-microcornea, cornea plana,
hypopigmentation and hypoplasia of retina
109. Wagner syndrome (hyaloideoretinal degeneration)-corneal degeneration, band-
shaped keratopathy, hyaloideoretinal degeneration, narrowing of retinal vessels, retinal
detachment, avascular preretinal membranes
110. Waldenström syndrome (macroglobulinemia syndrome)-crystalline corneal deposits,
keratoconjunctivitis sicca, retinal venous thrombosis, retinal microaneurysms, cotton-
wool spots
111. Weil disease (leptospirosis)-keratitis, retinitis
112. Werner syndrome (progeria of adults)-bullous keratitis, paramacular retinal
degeneration
113. Wiskott-Aldrich syndrome (sex-linked draining ears, eczematoid dermatitis, bloody
diarrhea)-corneal ulcers, retinal hemorrhages
114. Yersiniosis-corneal ulcer, retinal hemorrhages
115. Zellweger syndrome (cerebrohepatorenal syndrome)-corneal opacities, narrowing of
retinal vessels, retinal holes without detachment, tapetoretinal degeneration
116. Zieve syndrome (hyperlipemia hemolytic anemia-icterus syndrome)-cloudy cornea,
corneal ulcers, retinal lipemia
Arffa RC. Grayson's disease of the com ea. 3rd ed. St. Louis: Mosby-Yearbook. 1991.
RETINAL LESIONS ASSOCIATED WITH DEAFNESS
1. Alport syndrome (hereditary familial congenital hemorrhagic nephritis)

2. Alström disease-retinitis pigmentosa
3. Choroideremia, obesity, and congenital deafness
5. Dialinas-Amalric syndrome (deaf-mutism-retinal degeneration syndrome)
*6. Harada syndrome (Vogt-Koyanagi-Harada syndrome)
8. Hunter syndrome (MPS II)
9. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly
10. Norrie disease-mental retardation, X-linked retinal malformation, and hearing loss
*11. Refsum syndrome (phytanic acid storage disease)
12. Retinal vessel changes, muscular dystrophy, mental retardation, and hearing loss
13. Rubella (German measles)-cardiac disorders, cataract, salt-and-pepper pigmentation
14. Sanfilippo syndrome (MPS III. autosomal recessive)
*15. Syphilis-acquired or congenital
16. Usher syndrome (hereditary retinitis pigmentosa-deafness syndrome)
Ayazi S. Choroideremia obesity, and congenital deafness. Am J Ophthalmol 1981; 92:63-

69.
Millay RH, et al. Ophthalmologic and systemic manifestations of Alström's disease. Am J

Ophthalmol 1986; 102: 482-490.
SUBRETINAL FIBROSIS

2. Long-standing rhegmatogenous retinal detachment
3. Proliferative vitreoretinopathy
4. Vogt-Koyanagi-Harada disease
Kuo IC, et al. Subretinal fibrosis in patients with Vogt-Koyanagi-Harada disease.

Ophthalmology 2000; 107: 1721-1728.
Rathinam SR, et al. Vogt-Koyanagi-Harada syndrome after cutaneous injury.

EPIRETINAL MEMBRANES-MEMBRANES THAT GROW ON THE INNER

SURFACE OF THE RETINA
*1. After retinal photocoagulation, cryotherapy, or reattachment of retina

2. Following blunt or penetrating injuries
*3. Idiopathic
4. Nonproliferative retinal vascular disorders
*5. Proliferative retinopathies
6. Rhegmatogenous retinal detachment (see p. 468)
7. Sickle cell disease, including sickle cell C, sickle cell S, and sickle cell B with
thalassemia
8. Vitreous hemorrhage (see p. 424)
Carney MD, Jampol LM. Epiretinal membranes in sickle cell retinopathy. Arch
Ophthalmol 1987; 105:214-217.
Cherfan GM, et al. Nuclear sclerotic cataract after vitrectomy for idiopathic epiretinal
membranes causing macular pucker. Am J Ophthalmol 1991; 111:434-438.
Lansing MB, et al. The effect of pars plana vitrectomy and transforming growth factor-
beta without epiretinal membrane peeling on full-thickness macular holes.
LINEAR STREAKS PATTERN IN FUNDUS
*1. Angioid streaks (see p. 526-529)

2. Bird-shot retinochoroidopathy
3. Choroidal rupture
4. Demarcation lines
*5. Presumed ocular histoplasmosis syndrome-peripheral, parallel to equator
6. Migrating parasites
A. Botfly larvae
B. Trematodes
*7. Retinal and choroidal detachment
*8. Snail-track configuration of lattice degeneration
Fountain JA, Schloegel TF. Linear streaks of the equator in the presumed ocular
histoplasmosis syndrome. Arch Ophthalmol 1981; 99:246.
YELLOW -ORANGE LESIONS OF SUBRETINAL FUNDUS
1. Acute inflammatory lesions of pigment epithelium, choriocapillaris, and choroid

*2. Detachment of retinal pigment epithelium
3. Isolated pocket of subretinal fluid
4. Subretinal fluid following scleral buckling procedure
Avirs LR, Hilton GF. Lesions simulating serous detachments of the pigment epithelium.
Lobes LR, Grand MG. Subretinal lesions following scleral buckling procedure. Arch
Ophthalmol 1980; 98:680-683.
TALC RETINOPATHY-DRUG ADDICTS WHO INJECT DRUGS

INTRAVENOUSLY
1. Optic disc neovascularization (see p. 562-563)

2. Peripheral retinal neovascularization (see p. 514)
3. Vitreous hemorrhage (see p. 424)
O'Brien RJ, Schroedl BL. Talc retinopathy. Optom Vis Sci 1991; 68:54-57.
Tse DT, Ober RR. Talc retinopathy. Am J Ophthalmol 1980; 90:624-640.
CRYSTALLINE RETINOPATHY
1. Bietti crystalline dystrophy (Bietti disease)

2. Chronic retinal detachment
3. Cystinosis (cystine storage-aminoaciduria-dwarfism syndrome)
4. Gyrate atrophy with hyperornithemia (ornithine ketoacid aminotransferase deficiency)
5. Hyperoxaluria (oxalosis)
6. Nitrofurantoin therapy
7. Retinal pathology
8. Sjögren-Larson syndrome (oligophrenia-ichthyosis-spastic diplegia syndrome)
*9. Talc emboli
*10. Tamoxifen retinopathy
Ahmed I, et al. Crystalline retinopathy associated with chronic retinal detachment. Arch
Ophthalmol 1998; 116: 1449-1454.
Ibanez HE, et al. Crystalline retinopathy associated with long-term nitrofurantoin

therapy. Arch Ophthalmol 1994; 112:304-305.
Willemsen MA, et al. Juvenile macular dystrophy associated with deficient activity of
fatty aldehyde dehydrogenase in Sjögren-Larsson syndrome. Am J Ophthalmol 2000;
130:782-789.
PULFRICH STEREO-ILLUSION PHENOMENON

This condition involves central serous elevation of the macula with abnormal
latency of the visual-evoked potential.
* 1. Optic nerve disease - demyelinating optic neuropathy

2. Media opacity
3. Anisocoria
4. Macular disease
Greenberg HS. Visual-evoked responses. J Clin Neuro Ophthalmol 1981; 1:273.
Hofeldt AJ, et al. Pulfrich stereo-illusion phenomenon in serous sensory retinal

detachment of the macula. Am J Ophthalmol 1985; 100:576-580.
PARAFOVEAL TELANGIECTASIA
This type of retinal microvascular anomaly involves the parafoveal capillary
network as well as immediately adjacent vascular bed and is best demonstrated by
fluorescein angiography.
* 1. Carotid artery obstruction

*2. Diabetes mellitus usually bilateral
*3. Idiopathic
4. Localized form of Coats disease, usually unilateral
5. Small-branch venular occlusion
6. Small retinal capillary hemangioma, usually unilateral
7. Roentgenogram, irradiation
Gass JD, Oyakawa T. Idiopathic juxtafoveal telangiectasia. Arch Ophthalmol 1982;

100;769.
Millay RH, et al. Abnormal glucose metabolism and parafoveal telangiectasia. Am J

Ophthalmol 1986; 102:363-370.
HEREDITARY PEDIATRIC RETINAL DEGENERATIONS
1. Acquired
A. Juvenile retinitis pigmentosa
B. Early onset retinitis pigmentosa
(1) Autosomal dominant
(2) Autosomal recessive
(3) X-linked recessive
2. Congenital
A. Complicated Leber congenital amaurosis
(1) Multiple neurologic abnormalities
(2) Others
(3) Saldino-Mainzer syndrome
(4) Senior-Loken syndrome (tubulointerstitial nephropathy syndrome)
(5) Zellweger syndrome (cerebrohepatorenal syndrome of Zellweger)
B. Uncomplicated Leber congenital amaurosis
Foxman SG, et al. Classification of congenital and early onset retinitis pigmentosa. Arch
Ophthalmol 1985; 103: 1502-1506.
Nickel B, Hoyt CS. Leber's congenital amaurosis. Arch Ophthalmol 1982; 100:1089-
1092.
RETICULAR PATTERN OF DARK LINES IN FUNDUS
1. Granular pigmentary pattern of the peripheral fundus

2. Multiple drusen of peripheral fundus
3. Reticular degeneration of the pigment epithelium (peripheral)
4. Reticular pattern dystrophy of posterior fundus (Sjögren reticular dystrophy, Mesker
macroreticular dystrophy, pattern dystrophy of the retinal pigment epithelium, Doyne
honeycomb reticular degeneration)
5. Tapetochoroidal hypopigmentation
Gass JDM, et al. Drusen and disciform macular detachment and degeneration. Arch
Ophthalmol 1973; 90; 206-217.
Lewis H, et al. Reticular degeneration of the pigment epithelium. Ophthalmology

1985:92:1485-1495.
RETINAL PIGMENT EPITHELIAL TEARS

This condition involves a flat, uniform, crescent-shaped area of exposed choroid
of pigment epithelial elevation.
1. Acute retinal necrosis

*2. After laser photocoagulation
3. Along margin of retinal detachment
*4. Associated with pigment epithelial detachments
5. Spontaneous
6. Trauma
Fox GM, Blumenkranz M. Giant retinal pigment epithelial tears m acute retinal necrosis.
Am J Ophthalmol 1993; 116; 302-306.
Levin LA, et al. Retinal pigment epithelial tears associated with trauma. Am J
Ophthalmol 1991; 112:396-400.
Schoeppner G, et al. The risk of fellow eye visual loss with unilateral retinal pigment
epithelial tears. Am J Ophthalmol 1989; 108:683-685.
RETINAL PIGMENT EPITHELIAL FOLDS
1. Choroidal folds (see p, 530)

2, Pigment epithelial detachment
3. Retinal pigment epithelial tears (rips)
4. Retinal striae
*5. Subretinal neovascularization
Schatz H, et al. Retinal pigment epithelial folds associated with retinal pigment epithelial
detachment in macular degeneration. Ophthalmology 1990; 97:658-665.
Schoeppner G, et al. The risk of fellow eye visual loss with unilateral retinal pigment
epithelial tears. Am J Ophthalmol 1989; 108:683-685.
MIZUO PHENOMENON
This condition involves a change of color of the fundus from red in the dark-
adapted state to golden immediately or shortly after the onset of light.
1. Oguchi disease
2. X-liked juvenile retinoschisis
3. X-linked recessive cone dystrophy
de long PTV, et al. Mizuo phenomenon in X-linked retinoschisis. Arch Ophthalmol 1991;
109:1104-1 108.
Mizuo GA. A new discovery in dark adaptation in Oguchi's disease. Acta Sac
Ophthalmol Jpn 1913; 17:1148-1150.
Usui T, et al. Mizuo phenomenon observed by scanning laser ophthalmoscopy in a

patient with Oguchi disease. Am J Ophthalmol 2000; 130:359-361.
WHITE-DOT FOVEA
This is a ring-like lesion in the macula with numerous confluent white dots
arranged in a ring around the foveal margin,
1. Crystalline retinopathies
2. Epiretinal membrane with pseudohole
3. Gunn dots
4. Macular halo syndrome
5. Macular hole
6. Niemann-Pick disease
7. Vitreomacular fraction syndrome
16
Choroid
CONTENTS
Angioid streaks 526
Choroidal folds 530
Lesions confused with malignant melanoma 530
Choroidal hemorrhage 531
Choroidal detachment 532
Conditions simulating posterior uveitis or choroiditis 536
Choroiditis (posterior uveitis) 537
Conditions simulating posterior uveitis in children 538
Choroiditis (posterior uveitis) in children 538
Syndromes and diseases associated with uveitis 539
Chorioretinitis juxtapapillaris 553
Characteristics of granulomatous and nongranulomatous inflammation in posterior
uvea 552
Choroidal neovascularization 553
Ischemic infarcts of choroid (Elschnig spots) 554
Chorioretinal and choriovitreal neovascularization 554
Uveal effusion 555
Choroid coloboma 555
Choroidal ischemia 556
Pars planitis 556
Differential diagnosis of pars planitis 558
ANGIOID STREAKS
Angioid streaks are ruptures of Bruch membrane characterized
ophthalmoscopically by brownish lines surrounding the disc and radiating toward the
periphery.
1. AC hemoglobinopathy
2. Acanthocytosis (abetalipoproteinemia, Bassen-Kornzweeg syndrome)
3. Acromegaly
4. Acquired hemolytic anemia
5. Beta thalassemia minor
6. Calcinosis
7. Chronic congenital idiopathic hyperphosphatasemia
8. Chronic familial hyperphosphatemia
9. Cardiovascular disease with hypertension
10. Cooley anemia
11. Diffuse lipomatosis
12. Dwarfism
13. Epilepsy
14. Facial angiomatosis
15. Fibrodysplasia hyperelastica (Ehlers-Danlos syndrome)
16. François dyscephalic syndrome (Hallermann-Streiff syndrome)
17. Hemochromatosis
18. Hereditary spherocytosis
19. Hypercalcinosis
20. Idiopathic thrombocytic purpura
21. Lead poisoning
22. Myopia
24. Ocular melanocytosis
25. Optic disc drusen
26. Osteitis deformans (Paget disease)
27. Pituitary tumor
28. Previous choroidal detachment
*29. Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome)
30. Senile (actinic) elastosis of the skin
32. Sturge-Weber syndrome
33. Trauma
34. Tuberous sclerosis
35. Thrombocytopenic purpura
Aessopos A et al. Angioid streaks in Sickle-thalassemia Am J Ophthalmol 1994;

117:589-592.
Mansour AM. Is there an association between optic disc drusen and angioid streaks?
Graefes Arch Clin Exp Ophthalmol 1992; 230:595-596.
Extracted Table Angioid Streaks
CHOROIDAL FOLDS
Choroidal folds are folds of the posterior pole, at the level of the choroid, with
Hruby lens and pattern of alternating light lines on fluorescein angiography.
1. Choroidal tumor, such as a melanoma

2. Disciform degeneration
3. Exophthalmos
4. Graves disease (Basedow syndrome)
5. High hyperopia
6. Idiopathic-no underlying pathologic state
7. Infection of paranasal sinuses
8. Long-standing orbital inflammation
9. Massive cranioorbital hemangiopericytoma
*10. Ocular hypotony (see p. 325)
11. Orbital mass
12. Papilledema (see p. 593)
13. Posteriorly located choroidal detachment
14. Postoperative condition, such as scleral buckle
15. Primary retinal detachment
16. Subretinal neovascularization
17. Uveitis
Griebel SR, Kosmorsky GS. Choroidal folds associated with increased intracranial
pressure. Am J Ophthalmol 2000; 129:513-516.
Leventer DB. Frontoethmoidal mucoceles causing bilateral chorioretinal folds. Arch

Shields JA, et al. Clinicopathologic correlation of choroidal folds: secondary to massive

cranioorbital hemangiopericytoma. Ophthal Plast Reconstr Surg 1992; 8:62-68.
LESIONS CONFUSED WITH MALIGNANT MELANOMA
1. Ciliary body and choroid

A. Angioid streaks (see p. 526)
B. Choroiditis
C. Coats disease
D. Detachment
E. Leukemia and lymphoma
F. Limited choroidal hemorrhage
G. Lymphoid hyperplasia
H. Nodular hyperplasia
I. Sclerouveitis
J. Tumors
(1) Hemangioma
(2) Melanocytoma
(3) Meningioma
(4) Metastatic carcinoma, including lung
(5) Neurilemmoma
(6) Neurofibroma
(7) Neuroendocrine tumor
(8) Nevus
(9) Retinal oligodendroglioma
K. Uveal effusion
2. Optic-nerve head
A. Congenital crater
B. Melanocytoma
3. Retina
A. Chorioretinitis
B. Ciliary body and choroid
C. Disciform macular degeneration
D. Foreign body
E. Hemorrhagic macrocyst of retina
F. Lesions of pigment epithelium
G. Retinal detachment
(1) Macular
(2) More extensive
H. Retinoschisis
4. Scleral thickening as amyloidosis
5. Vitreous body
A. Abscess
B. Hemorrhages
Brannan SO, et al. A choroidal amyloid-rich neuroendocrine tumor. Arch Ophthalmol

1999; 117:1081-1083.
Eagle RC, et al. Choroidal metastasis as the initial manifestation of a pigmented

neuroendocrine tumor. Arch Ophthalmol 2000; 118:841-845.
Marek J, et al. Retinal oligodendroglioma. Am J Ophthalmol 1999:128:389-391.
Shields JA. Shields CL. Intraocular tumors: a text and atlas. Philadelphia: WB Saunders,
1992.
Simon CK, et al. Bilateral uveal effusion associated with scleral thickening due to
amyloidosis. Arch Ophthalmol 2000; 118:1293-1295.
CHOROIDAL HEMORRHAGE
1. Acute choroiditis
*2. After glaucoma filtering procedure (especially with Sturge- Weber syndrome)
3. Choroidal vascular aneurysm
4. Choroidal vascular sclerosis, such as senile macular degeneration with hemorrhage
(disciform degeneration of the macula)
5. General diseases
A. Arteriosclerosis
B. Blood dyscrasias
(1) Leukemia
(3) Purpura
(4) Thrombocytopenia
C. Diabetes mellitus (Willis disease)
D. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
E. Paget disease (osteitis deformans)
F. Valsalva maneuver
6. Myopia-accompanied by choroidal atrophy
7. Papilledema-rare
Boker T, Steinmetz R. Hyperopia and choroidal neovascularization. Ophthalmology

1994; 101:972.
Madreperla SA, et al. Choroidal hemangiomas. Ophthalmology 1997; 104:1773-1779.

VanMeurs JC, van der Bosch WA. Suprachoroidal hemorrhage following a Valsalva
maneuver. Arch Ophthalmol 1993; 111: 1025-1026.
CHOROIDAL DETACHMENT
Choroidal detachment can be differentiated from retinal detachment and tumor by
its solid appearance, smooth surface, and appearance of normal retinal vessels with color
unchanged and good transillumination.
*1. Acute ocular hypotony (see p. 325)

A. Myopia
B. Operative or perforating wounds, including those required for surgical
treatment of cataract, glaucoma, grafting of cornea, and retinal detachment
C. Severe uveitis with severe visual loss, intense ocular pain, unusually low
tension, and extremely deep anterior chamber in women
D. Yttrium-aluminum-garnet (YAG) laser cyclophotocoagulation
2. Inflammatory disease
A. Acute sinusitis
B. Chronic cyclitis
C. Harada disease (Vogt-Koyanagi-Harada syndrome)
D. Orbital abscess
E. Orbital pseudotumor
F. Scleritis and tenonitis
G. Sympathetic ophthalmia
3. Neoplastic disease
A. Intraocular tumor, such as metastatic or malignant melanoma
B. Leukemia
C. Orbital tumor
4. Spontaneous detachment associated with uveal effusion, such as nonrhegmatogenous
retinal detachment, shifting subretinal fluid, and peripheral annular choroidal detachment
affecting males almost exclusively
5. Trauma
A. Complication of scleral buckling retinal detachment surgery
B. Contusion of globe without perforation
C. Following perforation injury, including that because of perforating corneal
ulcer
D. Phthisical eye with traction of organized inflammatory tissue
6. Vascular disease
B. Disseminated intravascular coagulation
C. Hypertension
D. Leukemia
F. Nephritis
G. Oral acetazolamide
H. Periarteritis nodosa (Kussmaul disease)
I. Syphilitic vascular disease
J. Toxemia of pregnancy
Kurtz S, et al. Orbital pseudotumor presenting as acute glaucoma with choroidal and
retinal detachment. German J Ophthalmol 1993; 2:61-62.
Lakhanpal V. Experimental and clinical observations on massive suprachoroidal

hemorrhage. Trans Am Ophthalmol Soc 1993; 91:545-652.
Extracted Table Choroidal detachment
CONDITIONS SIMULATING POSTERIOR UVEITIS OR CHOROIDITIS
1. Angioid streaks (see p. 526)

2. Central serous retinopathy
A. Central serous retinopathy and exudative chorioretinopathy associated with
systemic vasculitis
B. Central serous retinopathy associated with crater-like holes in the optic disc
C. Choroidal
D. Chorioretinal
E. Retinal
3. Chorioretinopathy with hereditary microcephaly
4. Circinate retinopathy
5. Congenital macular dysplasia
6. Doyne homogeneous retinal degeneration
7. Drug-induced macular disease
A. Chloroquine (Aralen)
B. Indomethacin (Indocin)
C. Thioridazine (Mellaril)
8. Drusen because of the following:
A. Disease (vascular, inflammatory, or neoplastic)
B. Heredity (primary degeneration)
C. Senility
9. Fundus flavimaculatus
10. Helicoid peripapillary chorioretinal degeneration
11. Hemangioma of the choroid
12. Idiopathic hyperlipemia
13. Ischemic ocular inflammation
14. Ischemic optic neuropathy (vascular pseudopapillitis)
15. Jensen disease (juxtapapillary retinopathy)
16. Macular degeneration
18. Metastatic carcinoma
19. Night-blinding retinochoroidopathies
A. Predominantly choroidal heredodegenerations
(1) Choroidal sclerosis
(2) Choroideremia
(3) Fuchs spot
(4) Gyrate atrophy of choroid
(5) Myopic retinopathy and choroidopathy
B. Predominantly tapetoretinal heredodegenerations
(1) Retinitis pigmentosa group
(2) Retinitis punctata albescens
20. Opacities of the macular retina
A. Cotton-wool patches (see p. 491-494)
B. Glial scars
C. Hemorrhage
D. Hemosiderin
E. Inspissated exudates
F. Lipoid deposits
G. Pigment epithelium
(1) Pigment epithelium migration
(2) Pigment epithelium secretion
(3) Pigment epithelium seeds
(4) Proliferation in response to demand for phagocytes
(5) Proliferation with formation of cuticular masses
(6) Proliferation with metaplasia
(7) Simple proliferation
21. Peripheral chorioretinal atrophy
22. Pigmentary perivenous-chorioretinal degeneration
23. Primary familial amyloidosis
25. Retinal perforation during surgical treatment for strabismus
26. Retinal vasculitis
A. Involvement of central retinal vein (papillophlebitis)
B. Retinal periarteritis
C. Retinal periphlebitis
27. Retinoblastoma
28. Sickle cell retinopathy
29. Solar bums
30. Sorsby pseudoinflammatory (hemorrhagic) macular degeneration
31. Vitreous hemorrhage (see p. 421-422)
Schlaegel TF. Essentials of uveitis Boston: Little, Brown, 1969.
Suran A, et al. Immunology of the eye, workshop III. Immunologic aspects of ocular
disease: infection, inflammation, and allergy. Oxford: IRL Press. 1981.
CHOROIDITIS (POSTERIOR UVEITIS)

A. Herpes viruses
B. Peripheral uveitis (cyclitis)
C. Sarcoidosis syndrome (Schaumann syndrome)
D. Syphilis (acquired lues)
E. Toxoplasmosis
F. Tuberculosis
G. Unknown
H. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis syndrome)
2. Acquired immunodeficiency syndrome (AIDS)
3. Acute posterior multifocal placoid pigment epitheliopathy
4. Behçet syndrome
5. Bird-shot choroidopathy
6. Candidiasis
7. Cat-scratch disease
8. Cryptococcosis
9. Cytomegalovirus inclusion disease
11. Epstein-Barr virus
12. Lyme disease
13. Histoplasmosis
14. Multiple evanescent white dot syndrome
16. Pneumocystis carinii
17. Punctate inner choroidopathy
19. Serpiginous choroidopathy
22. Toxocariasis
*23. Toxoplasmosis
24. Unknown
25. Varicella zoster
Demiroglu H, et al. Risk factor assessment and prognosis of eye involvement in Behçet's
disease in Turkey. Ophthalmology 1997; 104:701-705.
Kerrison JR, et al. Retinal pathologic changes in multiple sclerosis. Retina 1994; 14:445-
451.
Ormerod LD, et al. Retinal and choroidal manifestations of cat-scratch disease.

Ophthalmology 1998; 105: 1024-1031.
Schubert HD, et al. Cytologically proven seronegative Lyme choroiditis and vitreitis.
Retina 1994; 14:39-41.
CONDITIONS SIMULATING POSTERIOR UVEITIS IN CIDLDREN
1. Coats syndrome (retinal telangiectasia)

2. Cockayne disease (dwarfism with retinal atrophy and deafness)
4. Hypogammaglobulinemia
5. Idiopathic hyperlipemia
6. Leukemia
7. Massive retinal fibrosis
*8. Retinoblastoma
Freidman AH, et al. An atlas of uveitis: diagnosis and management. Baltimore: Williams
& Wilkins, 1982.
Kraus M, et al. Uveitis - pathophysiology and therapy, 2nd ed. New York: Thieme, 1986.
CHOROIDITIS (POSTERIOR UVEITIS) IN CHILDREN
1. Ankylosing spondylitis
A. Sarcoidosis syndrome (Schaumann syndrome)
B. Sympathetic ophthalmia
C. Vogt-Koyanagi-Harada syndrome
3. Arteritis
4. Behçet disease (dermatostomatoophthalmic syndrome)
5. Chorioretinitis of unknown cause
A. Disseminated chorioretinitis
B. Juxtapapillary chorioretinitis
6. Cytomegalovirus inclusion disease (cytomegalovirus)
7. Diffuse unilateral subacute neuroretinitis
8. Herpes simplex chorioretinitis
9. Human immunodeficiency virus retinopathy
10. Inability of leukocytes to kill microorganisms
11. Intermediate uveitis
12. Juvenile psoriatic arthritis
14. Nematode (Toxocara) retinochoroiditis
15. Reiter disease (idiopathic blennorrheal arthritis syndrome)
16. Reticulum cell sarcoma of brain
17. Rubella
18. Subacute sclerosing panencephalitis
19. Syphilitic retinochoroiditis
*20. Toxoplasmic retinochoroiditis
21. Tuberculosis
Kanski JJ, Shun-Shin A. Systemic uveitis syndrome in childhood: an analysis of cases.

Ophthalmology 1984; 91: 1247-1251.
Kraus M, et al. Uveitis-pathophysiology and therapy, 2nd ed New York: Thieme, 1986.
Okada AA, Foster CS. Posterior uveitis in the pediatric population. Int Ophthalmol Clin
1992; 32:121-152.
SYNDROMES AND DISEASES ASSOCIATED WITH UVEITIS
1. Arthralgia
A. Hilding syndrome (destructive iridocyclitis and multiple joint dislocations)
B. Histoplasmosis
C. Whipple disease (intestinal lipodystrophy)
2. Arthritis
A. Ankylosing spondylitis
B. Behçet syndrome (dermatostomatoophthalmic syndrome)
C. Bleu syndrome ""
D. Familial histiocytic dermatoarthritis syndrome
E. Felty syndrome (rheumatoid arthritis with hypersplenism)
F. Gonorrhea
G. Juvenile rheumatoid arthritis (Still disease)
H. Leprosy (Hansen disease)
I. Mucocutaneous lymph node syndrome
J. Progressive systemic sclerosis
K. Psoriatic arthritis
L. Reiter syndrome (polyarthritis enteric a)
M. Rheumatoid arthritis (adult)
N. Sarcoidosis syndrome (Schaumann syndrome)
O. Sporotrichosis
P. Ulcerative colitis
Q. Van Metre peripheral polyarthritis or mono arthritis
R. Whipple disease (intestinal lipodystrophy)
S. Yersiniosis
3. Cataract
A. Acrodermatitis chronic a atrophicans
B. Andogsky syndrome (dermatogenous cataract)
C. Anterior segment ischemia syndrome
D. Arteriovenous fistula
E. Atopic dermatitis
F. Carotid artery syndrome (carotid vascular insufficiency syndrome)
G. Cerebral palsy
H. Chickenpox
I. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
J. Cytomegalic inclusion disease
K. Electrical injury
L. Familial histiocytic dermatoarthritis syndrome
M. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial
syndrome)
N. Herpes simplex
O. Herpes zoster
P. Hilding syndrome (destructive iridocyclitis and multiple joint dislocations)
Q. Histiocytosis X (Hand-Schüller-Christian syndrome)
R. Hodgkin disease (lymph node disease)
S. Homocystinuria syndrome
T. Hypervitaminosis D
U. Influenza
V. Juvenile rheumatoid arthritis (Still disease)
W. Kussmaul disease (periarteritis nodosa)
X. Leptospirosis (Weil disease)
Y. Listerellosis
Z. Malaria
AA. Measles (rubeola)
BB. Moniliasis (idiopathic hypoparathyroidism)
CC. Myotonic dystrophy syndrome
DD. Oculootoororenoerythropoietic disease
EE. Passow syndrome (syringomyelia)
FF. Radiation
GG. Relapsing polychondritis
HH. Rubella syndrome
II. Sarcoidosis syndrome (Schaumann syndrome)
JJ. Stevens-Johnson syndrome (erythema multiforme exudativum)
KK. Stickler syndrome (hereditary progressive arthroophthalmyopathy)
LL. Toxocariasis (visceral larva migrans syndrome)
MM. Toxoplasmosis
NN. Werner syndrome (progeria of adults)
OO. Yersiniosis
4. Conjunctivitis
A. Acanthamoeba
B. Actinomycosis
C. African eyeworm disease
D. Amebiasis
E. Andogsky syndrome (dermatogenous cataract)
F. Angular conjunctivitis
G. Ascariasis
H. Atopic dermatitis
I. Bacillary dysentery (shigellosis)
J. Bacterial endocarditis
K. Behçet syndrome (dermatostomato-ophthalmic syndrome)
L. Boutonneuse fever (rickettsia, Marseilles fever)
M. Brucellosis
N. Candidiasis
O. Charlin syndrome (nasal nerve syndrome)
P. Chlamydia
Q. Coccidioidomycosis
R. Coenurosis
S. Cogan syndrome (non syphilitic interstitial keratitis)
T. Crohn disease (granulomatous ileocolitis)
U. Cytomegalic inclusion disease
V. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
W. Epidermic keratoconjunctivitis
X. Escherichia coli
Y. Felty syndrome (rheumatoid arthritis with hypersplenism)
Z. Haemophilus aegyptius
AA. Herpes simplex
BB. Herpes zoster
CC. Hodgkin disease
DD. Infectious mononucleosis
EE. Influenza
FF. Kussmaul disease (periarteritis nodosa)
GG. Leptospirosis (Weil disease)
HH. Listerellosis
II. Lymphogranuloma venereum
JJ. Measles (rubeola)
KK. Meningococcemia
LL. Metastatic bacterial endophthalmitis
MM. Mikulicz-Radecki syndrome (dacryosialoadenopathy)
NN. Moniliasis (idiopathic hypoparathyroidism)
OO. Moraxella lacunata
PP. Mucocutaneous lymph node syndrome
QQ. Mumps
RR. Mycosis fungoides syndrome (Sézary syndrome)
SS. Nocardiosis
TT. Ocular vaccinia
UU. Pneumococcus
VV. Polymyositis dermatomyositis
WW. Progressive systemic sclerosis (scleroderma)
XX. Psoriatic arthritis
YY. Q fever
ZZ. Radiation
AAA. Reiter syndrome (polyarthritis enterica)
BBB. Relapsing fever
CCC. Rocky Mountain spotted fever
DDD. Rubella syndrome
EEE. St. Anthony fire (erysipelas)
FFF. Seborrheic dermatitis
GGG. Sporotrichosis
HHH. Staphylococcus
III. Stevens-Johnson syndrome (erythema multiforme exudativum)
JJJ. Streptococcus
KKK. Syphilis (acquired lues)
LLL. Trichinellosis
MMM. Tuberculosis
NNN. Vaccinia
OOO. Xeroderma pigmentosum
PPP. Yersiniosis
5. Cornea
A. Acanthamoeba
B. Acrodermatitis chronic atrophicans
C. Actinomycosis
D. African eyeworm disease
E. Andogsky syndrome (dermatogenous cataract)
F. Angioedema (Quincke disease)
G. Angular conjunctivitis (Morax-Axenfeld bacillus)
H. Ankylosing spondylitis
I. Anterior segment ischemia syndrome
J. Arteriovenous fistula
K. Atopic dermatitis
L. Bee sting of the cornea
M. Behçet syndrome (dermatostomatoophthalmic syndrome)
N. Brucellosis (Bang disease)
O. Candidiasis
P. Charlin syndrome (nasal nerve syndrome)
Q. Chickenpox
R. Chlamydia
S. Cockayne syndrome (dwarfism with retinal atrophy and deafness)
T. Cogan syndrome (I) (nonsyphilitic interstitial keratitis)
U. Crohn disease (granulomatous ileocolitis)
V. Cystinosis syndrome
W. Cytomegalic inclusion disease
X. Disseminated lupus erythematous (Kaposi-Libman-Sacks syndrome)
Y. Electrical injury
Z. Epidemic keratoconjunctivitis
AA. E. coli
BB. Felty syndrome (rheumatoid arthritis with hypersplenism)
CC. Gonorrhea
DD. H. aegyptius
EE. Herpes simplex
FF. Herpes zoster
GG. Hilding syndrome (destructive iridocyclitis and multiple joint dislocations)
HH. Histiocytosis X (Hand-Schüller-Christian syndrome)
II. Hodgkin disease (lymph node disease)
JJ. Homocystinuria syndrome
KK. Hypervitaminosis D
LL. Hypothermal injury
MM. Influenza
NN. Japanese River fever (mite borne typhus)
OO. Juvenile rheumatoid arthritis (Still disease)
PP. Juvenile xanthogranuloma (nevoxanthoendothelioma)
QQ. Kussmaul disease (periarteritis nodosa)
RR. Leprosy (Hansen disease)
SS. Leptospirosis (Weil disease)
TT. Lewis syndrome (tuberoserpiginous syphilid of Lewis)
UU. Listerellosis
VV. Lockjaw
WW. Lymphogranuloma venereum
XX. Malaria
YY. Measles (rubeola)
ZZ. Meningococcemia
AAA. Mikulicz-Radecki syndrome (dacryosialoadenopathy)
BBB. Moniliasis-idiopathic hypoparathyroidism (Addison syndrome)
CCC. Moraxella lacunata
DDD. Mumps
EEE. Mycosis fungoides syndrome (Sézary syndrome)
FFF. Myotonic dystrophy syndrome
GGG. Nocardiosis
HHH. Ocular vaccinia
III. Onchocerciasis syndrome
JJJ. Passow syndrome (status dysraphicus)
KKK. Plague
LLL. Pneumococcus
MMM. Postvaccinial ocular syndrome
NNN. Progressive systemic sclerosis (scleroderma)
OOO. Psoriasis
PPP. Psoriatic arthritis
QQQ. Radiation
RRR. Reiter syndrome (polyarthritis enterica)
SSS. Relapsing fever
TTT. Relapsing polychondritis
UUD. Rheumatoid arthritis (adult)
VVV. Rubella syndrome
WWW. St. Anthony fire (erysipelas)
XXX. Sarcoidosis syndrome (Schaumann syndrome)
YYY. Seborrheic dermatitis
ZZZ. Sporotrichosis
AAAA. Staphylococcus
BBBB. Stevens-Johnson syndrome (erythema multiforme exudativum)
CCCC. Stickler syndrome (hereditary progressive arthroophthalmopathy)
DDDD. Streptococcus
EEEE. Syphilis
FFFF. Thelaziasis
GGGG. Toxoplasmosis
HHHH. Tuberculosis
IIII. Vaccinia
JJJJ. Werner syndrome (progeria of adults)
KKKK. Xeroderma pigmentosum
LLLL. Yersiniosis
6. Diarrhea
A. Amebiasis
B. Bacillary dysentery
C. Chlamydia
D. Crohn disease (granulomatous ileocolitis)
E. E. coli
F. Listerellosis
G. Psoriatic arthritis
H. Regional enteritis (ulcerative colitis)
I. Rubella syndrome
J. Staphylococcus
K. Ulcerative colitis (regional enteritis)
L. Whipple disease (intestinal lipodystrophy)
M. Yersiniosis
7. Disc neovascularization
A. Ischemic uveitis of Knox
B. Papillophlebitis
8. Exophthalmus (proptosis)
A. Actinomycosis
B. Angioedema
D. Coenurosis
E. Cryptococcosis
F. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
G. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial
syndrome)
H. Herpes zoster
I. Histiocytosis X (Hand-Schüller-Christian syndrome)
J. Juvenile xanthogranuloma (nevoxanthoendothelioma)
K. Kussmaul disease (periarteritis nodosa)
L. Mumps
M. Polymyositis dermatomyositis
N. Relapsing polychondritis
O. Streptococcus
P. Trichinellosis
Q. Werner syndrome (progeria of adults)
9. Exudative detachment
A. Acute retinal necrosis syndrome
B. Bacterial endocarditis
C. Boutonneuse fever (rickettsia, Marseilles fever)
D. Cryptococcosis
E. Histiocytosis X (Hand-Schüller-Christian syndrome)
F. Japanese River fever (typhus)
G. Kussmaul disease (periarteritis nodosa)
H. Mycosis fungoides syndrome
I. Oculootoororenoerythropoietic disease
J. Pappataci fever (sandfly fever)
K. Periocular and ocular metastatic tumors
L. Progressive systemic sclerosis (scleroderma)
M. Rheumatic fever
N. Rocky Mountain spotted fever
O. Sarcoidosis syndrome (Schaumann syndrome)
P. Schwartz syndrome (glaucoma associated with retinal detachment)
Q. Stickler syndrome (hereditary progressive arthroophthalmopathy)
R. Toxocariasis (visceral larva migrans syndrome)
S. Toxoplasmosis
T. Weber-Christian syndrome (subcutaneous inflammatory lesions)
10. Glaucoma
A. Acanthamoeba
B. Angioedema
D. Acariasis
F. Behçet syndrome (dermatostomatoophthalmic syndrome)
G. Brucellosis (late manifestation) (Bang disease)
H. Carotid artery syndrome
I. Coats disease (retinal telangiectasia)
J. Coccidioidomycosis
K. Coenurosis
L. Electrical injury
M. E. coli
N. Familial histiocytic dermatoarthritis syndrome
O. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial
syndrome)
P. Homocystinuria syndrome
Q. Juvenile rheumatoid arthritis (Still disease)
R. Juvenile xanthogranuloma (nevoxanthoendothelioma)
S. Leprosy (Hansen disease)
T. Listerellosis
U. Measles (rubeola)
V. Oculootoororenoerythropoietic disease
W. Onchocerciasis syndrome
X. Periocular and ocular metastatic tumors
Y. Pneumococcus
Z. Posner-Schlossman syndrome (glaucomatocyclitic crisis)
AA. Pseudouveitis, glaucoma, hyphema syndrome (PUGH syndrome)
BB. Radiation
CC. Relapsing polychondritis
DD. Rubella syndrome
EE. Sarcoidosis syndrome (Schaumann syndrome)
FF. Schwartz syndrome (glaucoma associated with retinal detachment)
GG. Staphylococcus
HH. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)
II. Streptococcus
JJ. Trichinellosis
KK. Uveitis, glaucoma, hyphema syndrome (UGH syndrome)
LL. Weber-Christian syndrome (subcutaneous inflammatory lesions)
11. Hepatomegaly
A. Cytomegalic inclusions disease
B. Toxocariasis (visceral larva migrans syndrome)
C. Toxoplasmosis
12. Influenza-like disease
A. Acanthamoeba
B. Amebiasis
C. Bacillary dysentery
D. Boutonneuse fever (rickettsia, Marseilles fever)
E. Brucellosis (Bang disease)
F. Chickenpox
G. Chlamydia
H. E. coli
I. Gonorrhea
J. H. aegyptius
K. Histoplasmosis
L. Influenza
M. Japanese River fever (mite-borne typhus)
N. Leptospirosis (Weil disease)
O. Malaria
P. Measles (rubeola)
Q. Meningococcemia
R. Mumps
S. Pappataci fever (sandfly fever)
T. Plague (bubonic plague)
U. Pneumococcus
V. Q fever
W. Relapsing fever
X. Rocky Mountain spotted fever
Y. Staphylococcus
Z. Streptococcus
AA. Toxoplasmosis
BB. Tuberculosis
13. Iris neovascularization, such as Knox ischemic ocular inflammation (rubeosis iridis)
14. Jaundice
A. Leptospirosis
15. Meningism (meningitis)
A. Acanthamoeba
B. Behçet syndrome (dermatostomato-ophthalmic syndrome)
C. Cryptococcosis
D. Gonorrhea
E. Herpes simplex
F. Histoplasmosis
G. Leptospirosis (Weil disease)
H. Listerellosis
I. Meningococcemia
J. Sympathetic ophthalmia
K. Toxoplasmosis
L. Tuberculosis
16. Microphthalmia
A. Cytomegalic inclusion disease
B. Hallermann-Streiff-François syndrome (dyscephalic mandibulooculofacial
syndrome)
C. Mumps
D. Rubella syndrome
E. Toxoplasmosis
17. Nodules in the leg
A. Histoplasmosis
B. Sarcoidosis syndrome (Schaumann syndrome)
C. Ulcerative colitis
18. Optic neuritis (papillitis)
A. Angioedema
B. Behçet syndrome (dermatostomatoophthalmic syndrome)
D. Brucellosis (Bang disease)
E. Candidiasis
F. Chickenpox
G. Cytomegalic inclusion disease
H. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
I. Electrical injury
J. Felty syndrome (rheumatoid arthritis with hypersplenism)
K. Herpes zoster
L. Hodgkin disease (lymph node disease)
M. Infectious mononucleosis
N. Influenza
O. Juvenile rheumatoid arthritis (Still disease)
P. Leptospirosis
Q. Malaria
R. Measles
S. Meningococcemia
T. Mikulicz- Radecki syndrome (dacryosialoadenopathy)
U. Mumps
V. Ocular vaccinia
w. Onchocerciasis syndrome
X. Pappataci fever (sandfly fever)
Y. Postvaccinial ocular syndrome
Z. Q fever
AA. Reiter syndrome (polyarthritis enteric a)
BB. Regional enteritis (ulcerative colitis)
CC. Rocky Mountain spotted fever
DD. Sarcoidosis syndrome (Schaumann syndrome)
EE. Stevens-Johnson syndrome (erythema multiforme exudativum)
FF. Streptococcus
GG. Sympathetic ophthalmia
HH., Syphilis (acquired lues)
II. Toxocariasis (visceral larva migrans syndrome)
JJ. Toxoplasmosis
KK. Trichinellosis
LL. Tuberculosis
MM. Vaccinia
19. Papilledema
A. Angioedema
B. Arteriovenous fistula
C. Acariasis
D. Bacterial endocarditis
E. Behçet syndrome (dermatostomatoophthalmic syndrome)
F. Brucellosis (Bang disease)
G. Chickenpox
H. Coccidioidomycosis
I. Cryptococcosis
J. Cysticercosis
K. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
L. Histiocytosis X (Hand-Schüller-Christian syndrome)
M. Hodgkin disease (lymph node disease)
N. Hypervitaminosis D
O. Malaria
P. Moniliasis-idiopathic hypoparathyroidism (Addison disease)
Q. Mycosis fungoides syndrome (Sézary syndrome)
R. Papillophlebitis
S. Pappataci fever (sandfly fever)
T. Passow syndrome (status dysraphicus)
U. Periocular and ocular metastatic tumors
V. Progressive systemic sclerosis
W. Sarcoidosis syndrome (Schaumann syndrome)
X. Syphilis
Y. Trichinellosis
Z. Whipple disease (intestinal lipodystrophy)
20. Paralysis of extraocular muscle
A. African eyeworm disease
B. Arteriovenous fistula
C. Bacterial endocarditis
D. Brucellosis
E. Cerebral palsy
F. Chickenpox
G. Coccidioidomycosis
H. Cysticercosis
I. Disseminated lupus erythematosus
J. Electrical injury
K. Herpes simplex
L. Herpes zoster
M. Hodgkin disease (lymph node disease)
N. Hypothermal injury
O. Infectious mononucleosis
P. Influenza
Q. Kussmaul disease (periarteritis nodosa)
R. Lockjaw (tetanus)
S. Malaria
T. Measles (rubeola)
U. Meningococcemia
V. Multiple sclerosis
W. Mumps
X. Ocular vaccinia
Y. Passow syndrome (status dysraphicus)
Z. Periocular and ocular metastatic tumors
AA. Reiter syndrome (polyarthritis enterica)
BB. Relapsing fever
CC. Relapsing polychondritis
DD. Rocky Mountain spotted fever
EE. Streptococcus
FF. Syphilis (acquired lues)
GG. Trichinellosis
21. Perivenous sheathing
A. Acanthamoeba
B. Amebiasis
D. Brucellosis (Bang disease)
E. Candidiasis
F. Coccidioidomycosis
G. Metastatic bacterial endophthalmitis
H. Metastatic fungal endophthalmitis
I. Multiple sclerosis
J. Myotonic dystrophy syndrome
K. Ocular vaccinia
L. Onchocerciasis syndrome
M. Plague
N. Postvaccinial ocular syndrome
O. Q fever
P. Sarcoidosis syndrome (Schaumann syndrome)
Q. Syphilis (acquired lues)
R. Toxocariasis (visceral larva migrans syndrome)
S. Toxoplasmosis
T. Tuberculosis
U. Vaccinia
V. Weber-Christian syndrome (subcutaneous inflammatory lesions)
22. Pneumonitis
A. Chlamydia
B. Cytomegalic inclusion disease
C. Plague (Bubonic plague)
D. Pneumococcus
E. Rubella syndrome
F. Toxocariasis
G. Whipple disease (intestinal lipodystrophy)
23. Prostatitis
A. Gonococcosis
B. Whipple disease (intestinal lipodystrophy)
24. Salt-and-pepper fundus
A. Choroideremia in males
B. Cockayne disease (dwarfism with retinal atrophy and deafness)
C. Cystinosis
D. Prenatal influenza
E. Prenatal syphilis
25. Skin lesions
A. Acrodermatitis chronica atrophicans
B. African eyeworm disease
C. Andogsky syndrome (dermatogenous cataract)
D. Angioedema
F. Behçet syndrome (dermatostomato-ophthalmic syndrome)
G. Chickenpox
H. Disseminated lupus erythematosus (Kaposi-Libman-Sacks syndrome)
I. Familial histiocytic dermatoarthritis syndrome
J. Herpes simplex
K. Herpes zoster
L. Histiocytosis X (Hand-Schüller-Christian syndrome)
M. Juvenile xanthogranuloma (nevoxanthoendothelioma)
N. Leprosy (Hansen disease)
O. Lewis syndrome (tuberoserphiginous syphilid of Lewis)
P. Listerellosis
Q. Measles (rubeola)
R. Moraxella lacunata
S. Mucocutaneous lymph node syndrome
T. Mycosis fungoides syndrome (Sézary syndrome)
U. Polymyositis dermatomyositis
V. Postvaccinial ocular syndrome
W. Psoriasis
X. Psoriatic arthritis
Y. St. Anthony fire (erysipelas)
Z. Schistosomiasis (bilharziasis)
AA. Seborrheic dermatitis
BB. Sporotrichosis
CCC. Staphylococcus
26. Stomatitis
A. Behçet syndrome (dermatostomatoophthalmic syndrome)
B. Disseminated systemic histoplasmosis-not the ocular form
C. Herpes simplex
D. Lewis syndrome (tuberoserpiginous syphilid of Lewis)
E. Reiter syndrome (polyarthritis enteric a)
F. Regional enteritis
G. Ulcerative colitis
27. Tonsillitis
A. Whipple disease (intestinal lipodystrophy)
28. Trauma (nonpenetrating)
Friedman AH, et al. An atlas of uveitis: diagnosis and management. Baltimore: Williams
& Wilkins, 1982.
Kraus M, et al. Uveitis-pathophysiology and therapy, 2nd ed. New York: Thieme, 1986.
Extracted Table Chorioretinitis of granulomatous and nongranulomatous
inflammation in posterior uvea (???)
CHORIORETINITIS JUXTAPAPILLARIS
This large, irregular opaque mass that protrudes three to four diopters and
obscures the retinal vessels is seen near the disc and may be confused with acute optic
neuritis or a tumor.
1. Acanthamoeba keratitis of fellow eye

2. Bird-shot retinochoroidopathy
3. Coccidioides immitis
4. Histoplasmosis
7. Toxoplasmosis
8. Tuberculosis
Eide N. Skjeldal O. Juxtapapillary chorioretinitis in neurosyphilis. Acta Ophthalmol

1984; 62:351-358.
Haessler FH. Eye signs in general disease. Springfield, IL: Charles C Thomas, 1960:98.
John KJ, et al. Chorioretinitis in the contralateral eye of a patient with acanthamoeba
keratitis. Ophthalmology 1988; 95:635-639.
Woods AC. Endogenous inflammations of the uveal tract. Baltimore: Williams &
Wilkins, 1961.
CHOROIDAL NEOVASCULARIZATION
Choroidal neovascularization comprises new vessel formation from
choriocapillaris through a defect in the Bruch membrane as suggested by fluorescein
angiography.
1. Choroidal neovascular ingrowth at the margin of the optic nerve head

B. Hyaline bodies of optic nerve head
C. Idiopathic choroidal neovascularization
D. Macular drusen
E. Multiple evanescent white-dot and acute idiopathic blind spot enlargement
syndrome
F. Optic-nerve coloboma
G. Peripapillary choroiditis
H. Presumed ocular histoplasmosis syndrome
I. Pseudotumor cerebri (Symond syndrome)
J. Serpiginous choroiditis
2. Choroidal neovascular ingrowth through breaks in Bruch membrane in the macular
area
3. Acute posterior multifocal placoid pigment epitheliopathy
B. Behçet syndrome (dermatostomatoophthalmic syndrome) or Best disease
C. Bird-shot retinochoroidopathy
D. Choroidal rupture
E. Choroidal tumors
F. Chronic uveitis
G. Foveomacular dystrophy
H. Fundus flavimaculatus
I. Idiopathic choroidal neovascularization
*J. Macular drusen
K. Morning glory syndrome
L. Myopic degeneration
M. Parafoveal telangiectasis
N. Photocoagulation of macular lesions with argon laser
O. Presumed ocular histoplasmosis syndrome
P. Osteogenesis imperfecta (van der Hoeve syndrome)
Q. Retinitis pigmentosa
R. Rubella syndrome (Gregg syndrome)
S. Sarcoidosis syndrome (Schaumann syndrome)
T. Scars from previous deep macular hemorrhage
U. Senile disciform macular degeneration (Kuhnt-Junius disease)
V. Serpiginous choroiditis
W. Sorsby fundus dystrophy
X. Tilted disc syndrome
Y. Toxocariasis
Z. Toxoplasma retinochoroiditis
AA. Trauma
BB. Vein occlusion
CC. Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis
syndrome)
Callanan D, Gass JD. Multifocal choroiditis and choroidal neovascularization associated

with the multiple evanescent white dot and acute idiopathic blind spot enlargement
Dailey JR, et al. Peripapillary choroidal neovascular membrane associated with an optic
nerve coloboma. Arch Ophthalmol 1993; 111:441-442.
Feist RM, et al. Choroidal neovascularization in a patient with adult foveomacular

dystrophy and a mutation in the retinal degeneration slow gene. Am J Ophthalmol 1994;
118:259-260.
Lewis H, et al. Macular translocation for subfoveal choroidal neovascularization in age-
related macular degeneration: a prospective study. Am J Ophthalmol 1999; 128:135-146.
Shields JA, et al. Adenoma of the retinal pigment epithelium simulating a juxtapapillary
choroidal neovascular membrane. Arch Ophthalmol 2001; 119:289-292.
ISCHEMIC INFARCTS OF CHOROID (ELSCHNIG SPOTS)

When healed, these may show small, disseminated yellowish scars with central
pigment deposits and may be associated with retinal separation when acute.
1. Chronic glomerulonephritis
2. Collagen disease, such as scleroderma
*3. Malignant hypertension
4. Toxemia of pregnancy
Klein BA. Ischemic infarcts of choroid (Elschnig spots). Am J Ophthalmol 1968;

66:1069-1074.
Venecia G, et al. The eye in accelerated hypertension-Elschnig's spots in nonhuman

primates. Arch Ophthalmol 1980; 98:913.
CHORIORETINAL AND CHORIOVITREAL NEOVASCULARIZATION

This type of new vessel formation from choroid into the retina or vitreous usually
occurs after photocoagulation or after any of the following:
1. Atrophic scars in the presumed ocular histoplasmosis syndrome

4. Diseases of the retinal pigment epithelium
6. Leber syndrome (optic atrophy-amaurosis pituitary syndrome)
Chandra SR, et al. Choroidovitreal neovascular ingrowth after photocoagulation for

proliferative diabetic retinopathy. Arch Ophthalmol 1980; 98:1593.
Dizon-Moore RV, et al. Chorioretinal and choriovitreal neovascularization. Arch

UVEAL EFFUSION
Uveal effusion involves leaking of fluid from the choriocapillaris into the choroid
or subretinal space or both.
1. Hydrostatic
A. Dural arteriovenous fistula
*B. Hypotony, wound leak
C. Nanophthalmos
2. Idiopathic
3. Inflammatory
A. After panretinal photocoagulation
B. HIV
C. Scleritis, infected scleral buckle
D. Systemic lupus erythematosus
E. Trauma, intraocular surgery
F. Uveitis, sympathetic ophthalmia, Harada disease
Uyama M, et al. Uveal effusion syndrome. Ophthalmology 2000; 107:441-449.
Wisotsky BJ, et al. Angle closure glaucoma as an initial presentation of systemic lupus
erythematosus. Ophthalmology 1998; 105:1170-1172.
CHOROID COLOBOMA
1. Aicardi syndrome
2. Basal cell nevus syndrome (Gorlin syndrome)
3. Cat-eye syndrome (partial G-trisomy)
4. CHARGE association among coloboma, heart anomaly, choanal atresia, retardation,
genital and ear anomalies
5. Doubtful association
A. Crouzon syndrome (dysostosis craniofacialis)
B. Ellis-Van Crevald syndrome (chondroectodermal dysplasia)
C. Hallerman-Streiff syndrome (dyscephalic mandibulooculofacial)
D. Incontinentia pigmenti I (Block-Sulzberger syndrome)
E. Kartagener syndrome (bronchiectasis-dextrocardia-sinusitis)
F. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyl-
G. Pierre Robin syndrome (micrognathia-glossoptosis syndrome)
H. Stickler syndrome (hereditary progressive arthro-ophthalmopathy)
I. Tuberous sclerosis (Bourneville syndrome)
8. Isolated, sporadic
9. Joubert syndrome with bilateral chorioretinal coloboma (coloboma, chorioretinal with
cerebellar vermis aplasia)
10. Klinefelter syndrome (gynecomastia-aspermatogenesis syndrome)
11. Lenz microphthalmia syndrome
12. Linear sebaceous nevus syndrome
13. Median facial cleft syndrome
15. Retinal astrocytoma
17. Retinoblastoma
18. Rubinstein- Taybi syndrome (broad-thumbs syndrome)
19. Triploidy
*20. Trisomy (Edward syndrome)
21. Trisomy (Patau syndrome)
22. Turner syndrome
23. Warburg syndrome
24. 13q syndrome
Daufenbach DR, et al. Chorioretinal colobomas in a pediatric population. Ophthalmology

1998; 105:1455-1458.
Ward JR, et al. Upper-limb defect associated with developmental delay, unilateral poorly
developed
antihelix, hearing deficit, and bilateral choroid coloboma: a new syndrome. J Med Genet
1992; 29:589-591.
CHOROIDAL ISCHEMIA
This condition involves decreased choroidal perfusion demonstrated by
fluorescein angiography.
1. Arteritic anterior ischemic optic neuropathy

2. Disseminated intravascular coagulation
*3. Hypertension, severe
4. Renal failure
6. Toxemia of pregnancy
7. Thrombotic thrombocytopenic purpura
Kinyoun JL, Kalina RE. Visual loss from choroidal ischemia. Am J Ophthalmol 1986;
101:650-656.
Slavin ML, Barondes MJ. Visual loss caused by choroidal ischemia preceding anterior
ischemic optic neuropathy in giant cell arteritis. Am J Ophthalmol 1994; 117:81-86.
PARS PLANITIS (PERIPHERAL UVEITIS)

In pars planitis, inferior exudates in the peripheral retina, ora, pars plana, and
peripheral vitreous, vitreous ray and cells, posterior cortical cataract, perivasculitis,
partial thrombosis of central retinal vein, glaucoma, peripheral retinal hemorrhages, and
retinal detachment may be present.
1. Dental infection
2. Hereditary
*3. Idiopathic
5. Nematodiases
6. Rheumatic disease
8. Sinus infection
9. Streptococcal hypersensitivity
11. Toxoplasmosis
12. Ulcerative colitis (inflammatory bowel disease)
Josephberg RG, et al. A fluorescein angiographic study of patients with pars planitis and
peripheral exudation (snowbanking) before and after cryopexy. Ophthalmology 1994;
101:262-266.
Phillips WB, et al. Pars planitis presenting with vitreous hemorrhage. Ophthalmic Surg
Lasers 1993; 24:630-631.
Extracted Table Differential Diagnosis of Pars Planitis

17
Optic Nerve
CONTENTS
Blurred optic-nerve heads 559
Ciliooptic vein 559
Drusen of optic nerve 559
Fluid enlargement of retrobulbar optic nerve or sheath 560
Hyperemia of optic disc 560
Ischemic optic neuropathy 561
Linear hemorrhage on optic disc 562
Neovascularization of optic disc 562
Neuroretinitis 563
Optic-nerve atrophy 564
Optic-nerve atrophy and deafness 570
Syndromes and diseases associated with optic atrophy 571
Optic-nerve hypoplasia 577
Optic neuritis (papillitis and retrobulbar neuritis) 578
Pseudooptic neuritis 588
Opticociliary shunts 588
Papilledema 589
Pseudopapilledema 597
Peripapillary subretinal neovascularization 597
Pigmented tumors of optic disc 602
Pseudoglaucomatous atrophy of optic disc 602
Temporally displaced optic disc (dragged disc) 603
BLURRED OPTIC-NERVE HEADS
CILIOOPTIC VEIN
This vein appears at the disc edge and dips into the optic nerve to anastomose
with branches of the central retinal vein
1. Congenital
3. Sturge-Weber syndrome (meningocutaneous syndrome)
Zaret CR. et al. Cilio-optic vein associated with phakomatosis. Ophthalmology 1980;
87:330-334.
DRUSEN OF OPTIC NERVE

These white or yellow conglomerate, translucent bodies in the optic nerve may
cause field defects.
3. Angioid streaks (pseudoxanthoma elasticum; Grönblad-Strandberg syndrome)
4. Associated with corneal dystrophy
6. Friedreich ataxia
7. Glaucoma
8. Hereditary-autosomal dominant
9. High myopia
10. Idiopathic
11. Meningioma (unusual)
12. Pituitary tumor (unusual)
13. Retinal vein occlusion
15. Systemic lupus erythematosus (SLE)
16. Status dysraphicus syndrome (Passow syndrome)
19. Tuberous sclerosis (Bourneville syndrome)
Chern S, et al. Central retinal vein occlusion associated with drusen of the optic disc. Ann
Ophthalmol 1991; 23: 66-69.
1991.
Nischal KK, et al. Ocular ultrasound in Alagille syndrome. Ophthalmology 1997; 104:79-
85.
FLUID ENLARGEMENT OF RETROBULBAR OPTIC NERVE OR SHEATH

(DEMONSTRATED BY COMPUTED TOMOGRAPHIC SCANNING AND
ECHOGRAPHY)
1. Arachnoiditis
2. Basilar artery aneurysm
3. Bilateral temporal lobe cysts
4. Central retinal vein occlusion (see p. 468-472)
5. Facial trauma
6. Iliojejunal bypass
7. Occipital intradural arteriovenous malformation
8. Optic-nerve meningioma
9. Optic-nerve sheath cyst
10. Pseudotumor cerebri
12. Trauma (intrasheath hemorrhage of optic nerve)
13. Uveal meningeal syndrome
Hupp SL, Glaser JS. Optic nerve sheath decompression. Arch Ophthalmol 1987;
105:386-389.
HYPEREMIA OF OPTIC DISC
1. Central retinal vein thrombosis (see p. 468-472)

2. Hemangioma
3. Hypermetropia
4. Hypertensive retinopathy
5. Ischemic optic neuropathy
6. Neovascularization
7. Optic neuritis (see p. 578)
9. Polycythemia vera (Vaquez disease)
10. von Hippel-Lindau disease (retinocerebral angiomatosis)
Duke-Elder S. Scott GI. System of ophthalmology. vol 12. St. Louis: CV Mosby, 1971.
ISCHEMIC OPTIC NEUROPATHY

The anterior form is occlusive disease of the optic-nerve head and retrolaminar
region of the optic nerve; the posterior form is occlusion of one or more nutrient arteries
to the rest of the optic nerve. Onset is usually sudden, with painless unilateral visual loss
and visual-field defect.
1. Compression
A. Orbital hemorrhage (trauma)
B. Thyroid disease (Graves disease)
2. Drugs
A. Sumatriptan
B. Vigabatrin
3. Systemic diseases (often in combination)
A. Arteriosclerosis
B. Arrhythmia
C. Atherosclerosis
D. Cerebrovascular disease
E. Diabetes mellitus
F. Gastrointestinal ulcer
G. Hypercholesterolemia
H. Hyperhomocystinemia
I. Hyperparathyroidism
J. Hypertension, nocturnal hypotension
K. Ischemic heart disease
L. Sickle cell disease
M. Takayasu disease
4. Vasculitis
A. Allergic vasculitis
B. Buerger disease
C. Churg-Strauss syndrome (allergic granulomatosis and angiitis)
D. Collagen diseases, including polyarteritis nodosum and systemic lupus
E. Giant cell (temporal) arteritis
F. Postimmunization
G. Postviral vasculitis
H. Syphilis
5. Miscellaneous
A. Acute anemia
B. Anemia combined with hypotension
C. Carotid artery disease
D. Fabry disease (angiokeratoma corporis diffusum)
E. Hypertensive with peritoneal dialysis
F. Low tension glaucoma
G. Glucose-6-phosphate dehydrogenase deficiency
H. Migraine
I. Polycythemia vera
J. Radiation
K. Retinal artery occlusion
L. Tobacco
M. Various vascular disorders (e.g., cavernous sinus thrombosis)
Feldon SE. Anterior ischemic optic neuropathy: trouble waiting to happen.

Jackson TL, et al. Hypotensive ischemic optic neuropathy and peritoneal dialysis. Am J
Ophthalmol 1999; 128: 109-110.
Moster ML. Visual loss after coronary artery bypass surgery. Surv Ophthalmol 1998;
42:453-455.
Pianka P, et al. Hyperhomocystinemia in patients with nonarteritic anterior ischemic

optic neuropathy, central retinal artery occlusion and central retinal vein occlusion.
Ophthalmology 2000; 107:1588-1592.
Salomon O, et al. Analysis of prothrombotic and vascular risk factors in patients with
nonarteritic anterior ischemic optic neuropathy. Ophthalmology 1999; 106:739-742.
LINEAR HEMORRHAGE ON OPTIC DISC
2. Drusen of optic nerve
3. Glaucoma
A. Altitudinal field loss
B. Dense arcuate field loss
C. Sector-shaped field loss
5. Isolated finding
6. Leukemia
7. Systemic hypertension
Jonas JB, Xu L. Optic disk hemorrhages in glaucoma. Am J Ophthalmol 1994; 118:1-8.
Shihab ZM, et al. The significance of disc hemorrhage in open-angle glaucoma.

NEOVASCULARIZATION OF OPTIC DISC (GROWTH OF BLOOD VESSELS

ONTO OPTIC DISC)
1. Anemia
2. Age-related macular degeneration (AMD)
3. Arterial insufficiency
4. Behçet disease (oculobuccogenital syndrome)
7. Choroidal rupture
9. Drusen of optic nerve head
11. Geographic helicoid peripapillary choroidopathy
12. Glaucoma, chronic uncontrolled
13. Hereditary drusen of Bruch membrane
14. von Hippel-Lindau disease (retinocerebral angiomatosis)
15. Hypertensive retinopathy, advanced
16. Incontinentia pigmenti achromians (hypomelanosis of Ito syndrome)
17. Infection
A. Endophthalmitis
B. Congenital rubella syndrome (Gregg syndrome)
C. Histoplasmosis
D. Toxoplasmosis
18. Intraocular inflammation
A. Rheumatoid arthritis
B. Sarcoidosis syndrome (Schaumann syndrome)
C. Uveitis (unspecified)
19. Myopia, severe
21. Pseudotumor cerebri (Symond syndrome)
22. Retinal vein occlusion (see p. 468-472)
24. Retinopathy of prematurity (ROP)
26. Takayasu disease (aortic arch syndrome)
27. Trauma
28. Tumors
A. Benign
(1) von Hippel-Lindau disease
(2) Juxtapapillary capillary hemangioma
(3) Nevus
B. Malignant
(1) Choroidal melanoma
(2) Leukemia
(3) Lymphoma
(4) Metastatic tumors
29. Vogt-Koyangi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis)
563
Sanislo SR, et al. Corticosteroid therapy for optic disc neovascularization secondary to
chronic uveitis. Am J Ophthalmol 2000; 130:724-731.
To KW, et al. Bilateral optic disc neovascularization in association with retinitis

pigmentosa. Can J Ophthalmol 1991; 26:152-155.
NEURORETINITIS (INFLAMMATION OF OPTIC NERVE AND ADJACENT

RETINA)
1. Bartonella henselae infection

2. Cat-scratch disease
3. Herpes simplex
4. Idiopathic
5. Mumps (epidemic parotitis)
6. Nematode
7. Salmonella
8. Syphilis
9. Toxocara canis
10. Toxoplasmosis
Fish RH, et al. Toxoplasmosis neuroretinitis. Ophthalmology 1993; 100:1177-1182.
Gray AV, et al. Bartonella henselae inflection associated with peripapillary angioma,
branch retinal artery occlusion, and severe vision loss. Am J Ophthalmol 1999; 127,
2:223-224.
OPTIC-NERVE ATROPHY
A. Angelman syndrome (happy puppet syndrome; microdeletion of chromosome
15q11-13)
B. Chromosome deletion (long arm) syndrome (de Grouchy syndrome)
C. Cri-du-chat syndrome (cat-cry syndrome; deletion of short arm of chromosome
5)
D. Patau syndrome (trisomy syndrome)
E. Subacute sclerosing panencephalitis (Dawson disease)
2. Demyelinating and degenerative diseases
A. Arylsulfatase A deficiency syndrome (ADL, metachromatic leukodystrophy)
B. Devic syndrome (optical myelitis)
C. Hereditary motor sensory neuropathy (HMSN I; Charcot-Marie-Tooth
syndrome)
D. Hereditary optic atrophy (Behr syndrome)
E. Multiple sclerosis
3. Dermatologic disorders
A. Keratodermia palmaris et plantaris
B. McCune-Albright syndrome (fibrosus dysplasia)
C. Naegeli syndrome (reticular pigmented dermatosis)
D. Porphyria cutanea tarda
E. Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome)
F. Wrinkly-skin syndrome
4. Drugs, poisons, and vaccines, including the following:
acetophenazine barbital chloroquine
acetylsalicylic acid beclomethasone chlorpromazine
adrenal cortex injection benzathine penicillin G cholecalciferol
aldosterone betamethasone cisplatin
allobarbital bromide (?) clindamycin
amobarbital bupivacaine (?) cocaine
antimony lithium butabarbital colchicine compounds
thiomalate butalbital cortisone
antimony potassium butallylonal cyclobarbital
tartrate butaperazine cyclopentobarbital
antimony sodium butethal danazol
tartrate calcifediol dapsone
antimony sodium calcitriol desoxycorticosterone
thioglycollate carbamazepine dexamethasone
antipyrine carbon dioxide diatrizoate meglumine
aprobarbital carbromal or sodium
aspirin carphenazine diethazine
auranofin (?) cephaloridine (?) ergocalciferol
aurothioglucose (?) chloramphenicol (?) ethambutol
aurothioglycanide (?) chloroprocaine (?) ethopropazine
etidocaine (?) methylprednisolone sulfachlorpyridazine
fludrocortisone methyprylon sulfacytine
fluorometholone mitotane sulfadimethoxine
fluphenazine nadolol(?) sulfamerazine
fluprednisolone nalidixic acid sulfameter
gentamicin naproxen (?) sulfamethazine
glutethimide oxyphenbutazone sulfamethizole
gold Au 198 penicillamine sulfamethoxazole
gold sodium pentobarbital sulfamethoxypyridazine
thiomalate (?) perazine sulfanilamide
gold sodium pericyazine sulfaphenazole
thiosulfate (?) perphenazine sulfapyridine
heptabarbital phenelzine (?) sulfasalazine
hexachlorophene phenobarbital sulfathiazole
hexamethonium phenoxymethyl sulfisoxazole
hexethal penicillin sulthiame
hexobarbital phenylbutazone talbutal
hydrabamine piperacetazine tamoxifen
phenoxymethyl potassium penicillin G thiamylal
penicillin potassium penicillin V thiethylperazine
hydrocortisone potassium phenethicillin thiopental
hydroxychloroquine prednisolone thiopropazate
ibuprofen prilocaine (?) thioproperazine
interferon primidone thioridazine
iodine and iodine probarbital tobramycin
solutions and procaine (?) tranylcypromine (?)
iodochlor- procaine penicillin G triamcinolone
hydroxyquin procarbazine trichloroethylene
iodoquinol prochlorperazine trifluoperazine
isocarboxazid (?) promazine triflupromazine
isoniazid promethazine trimeprazine
lidocaine (?) propiomazine tryparsamide
medrysone propoxycaine (?) vaccines- influenza
mephobarbital quinine vinbarbital
mepivacaine (?) radioactive iodides vinblastine
meprednisone secobarbital vincristine
mesoridazine silicone vitamin A
methaqualone(?) sodium vitamin D (retinol)
metharbital antimonylgluconate vitamin D2
methdilazine sodium salicylate (ergocalciferol)
methitural stibocaptate vitamin D3
methohexital stibogluconate (cholecalciferol)
methotrimeprazine stibophen warfarin
methyl alcohol streptomycin
methylene blue sulfacetamide
5. Endocrine
A. Cretinism (hypothyroidism)
B. Cushing syndrome (adrenocortical syndrome)
C. Diabetes mellitus
D. DIDMOAD (diabetes mellitus and insipidus with optic atrophy and deafness)
syndrome; Wolfram syndrome, Marquardt-Loriaux syndrome)
E. Fröhlich syndrome (dystrophia adiposogenitalis)
F. Hyperparathyroidism
G. Hypophosphatasia (phosphoethanolaminuria)
H. Juvenile diabetes mellitus (Mauriac syndrome)
I. Pituitary gigantism syndrome (Launois syndrome)
J. Simmonds syndrome (hypopituitarism syndrome)
K. Retinohypophysis syndrome (Lijo Pavia-Lis syndrome)
L. Zollinger-Ellison syndrome (polyglandular adenomatosis syndrome)
6. Granulomatoses
A. Sarcoidosis
B. Tuberculosis
C. Wegener syndrome (Wegener granulomatosis)
7. Infectious
A. African eye-worm disease (loiasis)
B. Anthrax
C. Congenital cytomegalic inclusion disease
D. Congenital rubella syndrome (Gregg syndrome)
E. Cysticercosis
F. Deerfly fever (tularemia)
G. Encephalitis
H. Encephalomeningitis
I. Echinococcosis (hydatid cyst)
J. Lyme disease (borreliosis, relapsing fever)
K. Malaria
L. Meningitis
M. Measles (morbilli)
N. von Mikulicz-Radecki syndrome (dacryosialoadenopathy)
O. Mumps (epidemic parotitis)
P. Mycoplasma pneumoniae
Q. Onchocerciasis syndrome (river blindness)
R. Rocky Mountain spotted fever
S. Syphilis (congenital or acquired)
T. Toxoplasmosis
U. Yellow fever
8. Inherited
A. Congenital optic atrophy (autosomal dominant or recessive)
B. Jensen syndrome (opticoacoustic nerve atrophy with dementia; X-linked)
C. Juvenile optic atrophy (autosomal dominant)
D. Metaphyseal dysplasia, anetoderma and optic atrophy (autosomal recessive)
E. Myotonic dystrophy
F. Optic atrophy, cataract and neurologic disorder (dominant)
G. Optic atrophy, non-Leber type, early onset (X-linked)
H. Optic atrophy, polyneuropathy, and deafness (X-linked)
I. Optic atrophy, spastic paraplegia syndrome (X-linked)
J. Optic atrophy, spastic paraplegia, dementia (autosomal dominant)
K. Optic atrophy, nerve deafness, and distal neurogenic amyotrophy (recessive)
L. Optic atrophy with demyelinating of central nervous system (autosomal
dominant)
M. Optic atrophy hypoplasia, familial, bilateral (autosomal dominant)
9. Inherited metabolic disorders
A. Leukodystrophies
(1) Adrenoleukodystrophy (ALD)
(2) Canavan disease (spongy degeneration of the nervous system)
(3) Cockayne syndrome
(4) Homocystinuria syndrome
(5) Krabbe disease
(6) Maple syrup urine disease
(7) Menkes disease (kinky-hair syndrome)
B. Peroxisome abnormalities
(1) Defective biogenesis:
a. Infantile Refsum syndrome (heredopathia atactic a
polyneuritiformis)
b. Neonatal ALD (adrenal cortical atrophy, patchy brain
demyelination)
c. Zellweger syndrome (cerebrohepatorenal syndrome)
(2) Refsum syndrome (heredopathia atactica polyneuritiformis
(3) Rhizomeric chondrodysplasia punctata
(4) Single enzyme deficiency
a. Primary hyperoxaluria type I
b. X-linked ALD
C. Storage disorders
(1) Lipoidoses
a. Generalized gangliosidosis
(i) Gangliosidosis GM2, type
(ii) Generalized gangliosidosis GM type
(iii) Juvenile gangliosidosis GM type
b. Sphingolipidoses (arylsulfatase A deficiency syndrome
(i) Arylsulfatase A deficiency syndrome (metachromatic
leukodystrophy)
(a) Late infant form: Greenfield disease
(b) Adult form: Bogaert-Nijssen-Peiffer syndrome
(c) Austin disease (multiple sulfatase deficiency
(d) Fabry disease (angiokeratoma corporis
diffusum)
(e) Krabbe disease (globoid cell leukodystrophy)
(f) Niemann-Pick syndrome (essential lipoid
histiocytosis)
(g) Tay-Sachs syndrome (hexosaminidase
deficiency)
(2) Glucose-phosphate dehydrogenase deficiency (von Gierke disease)
(3) Mucolipidoses IV (ML IV)
(4) Mucopolysaccharidoses (MPS) or lysosomal storage diseases
a. MPS I-H (Hurler syndrome; chondroosteodystrophy or
lipochondrodystrophy)
b. MPS I-S (Scheie syndrome)
c. MPS II (Hunter syndrome)
d. MPS III (Sanfilippo syndrome)
e. MPS IV (Morquio syndrome)
f. MPS VI (Maroteaux-Lamy syndrome)
(5) Neural ceroid lipofuscinosis
a. Infantile type: Haltia-Santavuori disease
b. Late infantile type: Jansky-Bielschowsky disease (internuclear
ophthalmoplegia)
c. Juvenile type: Batten disease (Spielmeyer-Vogt-Sjögren
syndrome; cerebroretinal degeneration)
(6) Other disorders involving lipids
a. Bassen-Kornzweig syndrome (familial hypolipoproteinemia)
b. Refsum syndrome (heredopathia atactica polyneuritiformis)
10. Local
A. Aphakic cystoid macula edema (ACME; Irvine-Gass syndrome)
B. Bird-shot chorioretinopathy
C. Coats disease
D. Drusen of optic nerve
E. Glaucoma
F. Vascular occlusion
11. Mental and psychomotor deficiency, retardation
A. Drummond syndrome (idiopathic hypercalcemia)
B. Familial dysautonomia (Riley-Day syndrome)
C. Hallervorden-Spatz syndrome
D. Hallgren syndrome (retinitis pigmentosa-deafness-ataxia syndrome)
E. Kloepfer disease
F. Rubinstein-Taybi syndrome
12. Miscellaneous
A. Albinism
B. Anemia
C. Arachnoidal adhesion (e.g., caused by tabes)
D. Bloch-Sulzberger disease (incontinentia pigmenti)
E. Bobble-head doll syndrome (massive dilatation of third ventricle)
F. Bonnet-Dechaume-Blanc syndrome (neuroretinoangiomatosis)
G. Brown-Sequard syndrome
H. Cerebellar ataxia (Louis-Bar syndrome)
I. Cerebral palsy
J. Cystic fibrosis syndrome
K. Foster-Kennedy syndrome (basal-frontal syndrome)
L. Histiocytosis X eosinophil granuloma (Hand-Schuller-Christian syndrome)
M. Incipient prechiasmal optic nerve compression syndrome
N. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-
O. Leber syndrome
P. Oculodental syndrome (Peter syndrome)
Q. Optic cochleodental degeneration syndrome
R. Peliazeus-Merzbacher disease (aplasia axialis extracorticalis congenita)
S. Posthypoxic syndrome
T. Pseudotumor cerebri
U. Rieger syndrome
V. Russell syndrome
W. Status dysraphicus syndrome (Passow syndrome)
X. Sphenomaxillary fossa syndrome (pterygopalatine fossa syndrome)
Y. Wagner disease (hereditary vitreoretinal degeneration)
13. Nutritional deficiency
A. Avitaminosis B (Wernicke syndrome, beriberi)
B. Avitaminosis B (pellagra)
C. Garland syndrome (central nervous system deficiency)
D. (?) Infantile neuroaxonal dystrophy (possible vitamin E deficiency,
Seitelberger disease II)
E. Kwashiorkor syndrome (hypoproteinemia syndrome)
F. (?) Oculoorogenital syndrome (avitaminosis B with possible avitaminosis A)
14. Orbital
A. Hutchinson-Pepper syndrome (metastatic infraorbital neuroblastoma)
B. Rollet syndrome (orbital apex syndrome)
C. Superior orbital fissure syndrome (Rochon-Duvigneaud syndrome)
15. Rheumatoid
A. von Bechterew-Stumpelld syndrome (ankylosing spondylitis)
B. Behçet disease (oculobuccogenital syndrome)
C. Polymyalgia rheumatica
D. SLE
16. Skeletal disorders
A. Achondroplasia
B. Albers-Schonberg syndrome (osteopetrosis)
C. Anencephaly
D. Apert syndrome (acrocephalosyndactylism syndrome)
E. Brachmann-de Lange syndrome
F. Camurati-Engelmann syndrome (progressive diaphyseal dysplasia)
G. Chondrodystrophia calcificans congenita (Conradi syndrome)
H. Cloverleaf skull syndrome (Kleeblattschädel deformity)
I. Craniometaphyseal dysplasia (Pyle syndrome)
J. Craniostenosis
(1) Oxycephaly
(2) Plagiocephaly
(3) Scaphocephaly
(4) Trigonocephaly
K. Crouzon syndrome (craniofacial dysotosis)
L. Enchondromatosis syndrome (Ollier syndrome)
M. Generalized gangliosidosis GM type
N. Greig syndrome (hypertelorism ocularis)
O. Hallermann-Streiff-François syndrome (oculomandibulofacial dyscephaly)
P. Hutchinson-Gilford progeria syndrome (progeria)
Q. Marchesani syndrome
R. McCune-Albright syndrome (fibrosus dysplasia)
S. Metaphyseal dysplasia, anetoderma, and optic atrophy
T. Microcephaly
U. Osteogenesis imperfecta (van der Hoeve syndrome)
V. Paget syndrome (osteitis deformans)
W. Primary hyperoxaluria type (osteodystrophy hydrocephalus)
X. Zellweger syndrome (cerebrohepatorenal syndrome)
17. Trauma
A. Direct and indirect optic nerve trauma
B. Electrical injury
C. Mechanical injury/surgical trauma (orbital floor fracture, malar fractures,
Krönlein lateral orbitotomy)
D. Ocular contusion
E. Optic-nerve evulsion
F. Radiation
18. Tumors
A. Craniopharyngiomas
B. Ectopic pinealomas
C. Gliomas
D. Hemangiomas
E. Meningiomas
F. Nasopharyngeal carcinomas
G. Neuroblastomas
H. Pituitary adenomas
I. Pseudo-Foster Kennedy syndrome
J. Pseudo-pseudo-Foster Kennedy syndrome
K. von Recklinghausen syndrome (neurofibromatosis)
L. Tumors extending into fourth ventricle and cerebellum causing papilledema
19. Vascular
A. Aneurysm of internal carotid artery (foramen lacerum syndrome)
B. Arteriosclerosis
C. Cavernosus sinus thrombosis (Foix syndrome)
D. Giant cell (temporal arteritis)
E. Hollenhorst syndrome (chorioretinal infarction syndrome)
F. Kussmaul disease (necrotizing angiitis)
G. Occlusion of the carotid artery
H. Sickle cell disease (Herrick syndrome)
I. Takayasu syndrome (aortic arch syndrome)
Woburn, MA: Butterworth-Heinemann, 2001.
Gamez J, et al. Bilateral optic nerve atrophy in myotonic dystrophy. Am J Ophthalmol

2001; 131:398-400.
Girkin CA, et al. Radiation optic neuropathy after stereotactic radiosurgery.

Ophthalmology 1997; 104:1634-1643.
Swartz N, Savino PJ. Is all nondefinable optic atrophy Leber's hereditary optic
neuropathy? Surv Ophthalmol 1994; 39:146-150.
Optic-Nerve Atrophy and Deafness
1. Adult form of arylsulfatase A deficiency (Bogaert-Nijssen-Peiffer syndrome;

opticocochleodental degeneration)
2. Camuati-Engelmann syndrome (progressive diaphyseal dysplasia)
3. HMSN I (Charcot-Marie-Tooth syndrome)
4. Congenital rubella syndrome (German measles, Gregg syndrome)
6. Craniometaphyseal dysplasia (Pyle syndrome)
7. DIDMOAD syndrome (optic atrophy, sensorineural deafness, diabetes mellitus and
diabetes insipidus)
8. Dominant inheritance-congenital deafness and progressive optic nerve atrophy
9. Friedreich ataxia (optic atrophy, ataxia, and progressive hearing loss)
10. Generalized gangliosidosis GM type
12. Juvenile diabetes mellitus
13. Krabbe syndrome (infantile globoid [II] cell leukodystrophy)
14. MLIV
15. MPS I-H (Hurler syndrome)
16. MPS II (Hunter syndrome)
17. MPS IV (Morquio syndrome)
18. (?) MPS (Maroteaux-Lamy syndrome)
19. (?) Niemann-Pick syndrome (essential lipoid histiocytosis)
20. Osteogenesis imperfecta
21. Recessive: nerve deafness, optic atrophy, and distal neurogenic amyotrophy
22. Refsum syndrome (phytanic acid oxidase deficiency)
23. Rosenberg-Chutorian syndrome
24. Sylvester disease
25. Treft syndrome
Emery JM, et al. Krabbe's disease. Am J Ophthalmol 1972; 74:400-406.
Shields JA, et al. Pigmented adenoma of the optic nerve head simulating a
melanocytoma. Ophthalmology 1992; 99:1705-1709.
Syndromes and Diseases Associated with Optic Atrophy
1. Achondroplasia
2. Acquired lues (syphilis)
3. African eye worm disease (loiasis)
4. Albers-Schonberg syndrome (osteopetrosis)
5. Albinism
6. Albright syndrome (fibrous dysplasia)
7. Anemia
8. Anencephaly
9. Aneurysm of internal carotid artery syndrome (foramen lacerum syndrome)
10. Anthrax
12. Arachnoidal adhesion, including tabes
13. Arteriosclerosis
14. Arylsulfatase A deficiency syndrome (metachromatic leukodystrophy)
15. Avitaminosis B2 (pellagra)
17. Batten-Mayou syndrome (cerebroretinal degeneration)
19. Behr syndrome (optic atrophy-ataxia)
20. Bielschowsky-Jansky disease (internuclear ophthalmoplegia)
21. Bloch-Sulzberger disease (incontinentia pigmenti)
22. Bobble-head doll syndrome
23. Bonnet-Dechaume-Blanc syndrome (neuroretinoangiomatosis)
25. Brown-Sequard syndrome (lesion of spinal cord)
26. Carbon monoxide
27. Central nervous system deficiency-bitemporal pallor because of deficient diet
(Garland syndrome)
28. Cerebral palsy
29. Cerebellar ataxia
30. Charcot-Marie-Tooth syndrome (progressive neuritic muscular syndrome)
32. Chromosome deletion (long-arm) syndrome (de Grouchy syndrome)
35. Congenital cytomegalic inclusion disease
36. Congenital optic atrophy-autosomal dominant or recessive
37. Congenital syphilis
38. Craniometaphyseal dysplasia (Pyle syndrome)
39. Craniostenosis (including oxycephaly, scaphocephaly, trigonocephaly, and
plagiocephaly)
42. Crouzon syndrome (craniofacial dysostosis)
43. Cushing syndrome (adrenocortical syndrome)
44. Cystic fibrosis syndrome (fibrocystic disease of pancreas)
45. Cysticercosis
47. Deerfly fever (tularemia)
49. Devic syndrome (optical myelitis)
51. Didmoad -Wolfram syndrome (diabetes mellitus and insipidus with optic atrophy and
deafness )-autosomal recessive
52. Diencephalic syndrome (Penfield syndrome)
54. Dollinger-Bielschowsky syndrome (lipidosis)
acetophenazine bromisovalum cyclopentobarbital
allobarbital broxyquinoline cycloserine (?)
alseroxylon (?) bupivacaine (?) dapsone
aminosalicylate (?) butabarbital deferoxamine
aminosalicylic acid (?) butalbital deserpidine(?)
amobarbital butallylonal dexamethasone
amodiaquine butaperazine dextrothyroxine (?)
antimony lithium, butethal diethazine
thiomalate calcitriol digitalis (?)
antimony potassium carbromal diiodohydroxyquin
tartrate carphenazine ergocalciferol
antimony sodium chloramphenicol ergonovine (?)
tartrate chloroprocaine (?) ergot (?)
antimony sodium chloroquine ergotamine (?)
thioglycollate chlorpromazine ethambutol
antipyrine cholecalciferol ethopropazine
aprobarbital clindamycin etidocaine (?)
aspirin cobalt (?) ferrocholinate (?)
barbital cocaine ferrous fumarate (?)
betamethasone cortisone ferrous gluconate (?)
bromide (?) cyclobarbital ferrous succinate (?)
ferrous sulfate (?) oral contraceptives sulfadimethoxine (?)
fluorometholone oxyphenbutazone sulfamerazine (?)
fluphenazine pentobarbital sulfameter (?)
gentamicin perazine sulfamethazine
heptabarbital pericyazine sulfamethizole (?)
hexachlorophene perphenazine sulfamethoxazole (?)
hexamethonium phenobarbital sulfamethoxypyridazine
hexethal phenylbutazone sulfanilamide (?)
hexobarbital piperacetazine sulfaphenazole
hydrocortisone polysaccharide iron sulfapyridine (?)
hydroxychloroquine complex (?) sulfasalazine (?)
iodide and iodine prednisolone sulfathiazole (?)
solutions and prilocaine (?) sulfisoxazole (?)
compounds primidone suramin
iodochlorhydroxyquin probarbital syrosingopine (?)
iodoquinol procaine (?) talbutal
iron dextran (?) prochlorperazine thiamylal
iron sorbitex promazine thiethylperazine
isoniazid promethazine thiopental
levothyroxine (?) propiomazine thiopropazate
lidocaine (?) propoxycaine (?) thioproperazine
liothyronine propoxyphene thioridazine
liotrix (?) quinine thyroglobulin (?)
medrysone radioactive iodides thyroid (?)
mephobarbital rauwolfia serpentina (?) tobramycin
mepivacaine (?) rescinnamine (?) trichloroethylene
mesoridazine reserpine (?) trifluoperazine
metharbital secobarbital triflupromazine
methdilazine sodium trimeprazine
methitural antimonylgluconate tryparsamide
methohexital sodium salicylate vinbarbital
methotrexate (?) stibocaptate vinblastine
methotrimeprazine stibogluconate vincristine
methyl alcohol stibophen vitamin A
methylene blue streptomycin vitamin D
methylergonovine (?) sulfacetamide (?) vitamin D2
methylprednisolone sulfachlorpyridazine (?) vitamin D3
methysergide (?) sulfacytine (?)
nitroglycerin (?) sulfadiazine (?)

58. Dyschondroplasia syndrome (Ollier syndrome)
62. Exfoliation syndrome
63. Foix syndrome (cavernous sinus thrombosis)
64. Foster-Kennedy syndrome (basal-frontal syndrome)
65. Friedreich ataxia (optic atrophy and sensorineural deafness)-recessive
66. Fröhlich syndrome (dystrophia adiposogenitalis)
67. Galactosyl ceramide lipidosis (globoid cell leukodystrophy)
68. Gangliosidosis GM 1, type
69. Generalized gangliosidosis (infantile)
70. Greig syndrome (hypertelorism ocularis)
71. Glaucoma
72. Glucose-phosphate dehydrogenase deficiency (von Gierke disease)
73. Grönblad-Strandberg syndrome (systemic elastodystrophy)
74. Hallermann-Streiff-François syndrome (oculomandibulofacial dyscephaly)
76. Hallgren syndrome (retinitis-pigmentosa deafness-ataxia syndrome)
77. Happy-puppet syndrome (puppet children)
78. Herrick syndrome (sickle cell disease)
82. Hutchinson syndrome (progeria)
83. Hydatid cyst
84. Hydrocephalus chondrodystrophicus congenita (Kleeblattschädel syndrome)
87. Incipient prechiasmal optic nerve compression syndrome
88. Infantile neuroaxonal dystrophy (Seitelberger disease II)
89. Infantile type of neuronal ceroid lipofuscinosis
90. Infections such as basal meningitis, infectious encephalomeningitis (especially
measles epidemic parotitis), congenital neurosyphilis (rare before 2 years of age), and
toxoplasmosis
91. Irvine syndrome (spontaneous rupture of vitreous face with vitreous adhesions to the
wound followed by macular edema)
92. Jensen syndrome (opticoacoustic nerve atrophy with dementia)-X-linked
93. Juvenile diabetes-rare
94. Juvenile optic atrophy-autosomal dominant
95. Keratodermia palmaris et plantaris
96. Kloepfer disease
97. Krabbe disease (meningocutaneous syndrome)
99. Kwashiorkor (hypoproteinemia syndrome)
101. Leber syndrome (optic atrophy-amaurosis-pituitary syndrome)
103. Leukemia
104. Malaria
105. Maple syrup urine disease (branched-chain ketoaciduria)
106. Marchesani syndrome (brachymorphy with spherophakia)
107. Maroteaux-Lamy disease (MPS VI)
108. Marquardt-Loriaux syndrome
109. Measles (morbilli)
110. Menkes disease (kinky-hair disease)
111. MERRF syndrome
112. Metachromatic leukodystrophy (Greenfield disease)
113. Metaphyseal dysplasia, anetoderma, and optic atrophy-autosomal recessive
114. Microcephaly
115. Micro syndrome
116. Mikulicz-Radecki syndrome (dacryosialoadenopathy)
117. ML IV
118. MPS IH (Hurler syndrome)
119. MPS IS (Scheie syndrome)
120. MPS II (Hunter syndrome)
121. MPS IV (Morquio syndrome)
123. Mumps
124. Naegeli syndrome (reticular pigmented dermatosis)
125. Niemann-Pick syndrome (essential lipoid histiocytosis)
126. Occlusion of carotid artery
128. Oculoorogenital syndrome (riboflavin deficiency syndrome)
129. Onchocerciasis syndrome (river blindness)
130. Optic atrophy, cataract and neurologic disorder-dominant
131. Optic atrophy, nerve deafness, and distal neurogenic amyotrophy-recessive
132. Optic atrophy, non-Leber type, with early onset-x-linked
133. Optic atrophy, polyneuropathy and deafness-x-linked
134. Optic atrophy, spastic paraplegia syndrome-x-linked
135. Optic atrophy with demyelinating of central nervous system-autosomal dominant
136. Optic nerve hypoplasia, familial, bilateral-autosomal dominant
137. Opticocochleodental degeneration syndrome
138. Orbital operation, such as following orbital floor fracture, reduction of malar
fractures and Krönlein lateral orbitotomy
140. Passow syndrome (syringomyelia)
141. Pelizaeus-Merzbacher disease (aplasia axialis extracorticalis congenita)
142. Pituitary gigantism syndrome (Launois syndrome)
145. Posthypoxic encephalopathy syndrome
146. Radiation
148. Relapsing fever
149. Retinohypophysary syndrome (Lijo Pavia-Lis syndrome)
151. Riley-Day syndrome (congenital familial dysautonomia)
153. Rocky Mountain spotted fever
155. Rosenberg-Chutorian syndrome
156. Rubella syndrome (German measles)
157. Rubinstein-Taybi syndrome (broad thumb syndrome)
158. Russell syndrome
160. Sanfilippo disease (MPS III)
162. Schaumann syndrome (sarcoidosis syndrome)
163. Schilder syndrome (encephalitis periaxialis diffusa)
164. Simmonds syndrome (hypopituitarism syndrome)
165. Spastic paraplegia, optic atrophy, dementia-autosomal dominant
166. Sphenomaxillary fossa syndrome (pterygopalatine fossa syndrome)
167. Spielmeyer-Vogt syndrome (cerebroretinal degeneration)
168. Spongy degeneration of the white matter
169. Suprarenal-sympathetic syndrome (adrenal medulla tumor syndrome)
170. Sylvester disease
171. Simmond syndrome (benign intracranial hypertension)
172. Takayasu syndrome (aortic arch syndrome)
173. Tay-Sachs syndrome (hexosaminidase deficiency)
174. Temporal arteritis syndrome (Hutchinson-Horton-Magath-Brown syndrome)
175. Toxins, including lead, chronic cyanide intoxication such as from eating cassava,
thallium (used for treatment of scalp fungi)
176. Trauma, evulsion of optic nerve, and ocular contusion
177. Treft syndrome
178. Trisomy D syndrome (Patau syndrome)
179. Tuberculosis
180. Tumors, including craniopharyngiomas, ectopic pinealomas, gliomas, hemangiomas,
meningiomas, nasopharyngeal carcinomas, neuroblastomas, pituitary adenomas, and
tumors extending into fourth ventricle and cerebellum causing papilledema
181. Tunbridge-Paley disease
182. Vaccinia
183. Vascular accident
184. von Bekterev-Strumpell syndrome (ankylosing spondylitis)
188. Wernicke syndrome (thiamine deficiency)
189. Wrinkly-skin syndrome
190. Yellow fever
192. Zollinger-Ellison syndrome (polyglandular adenomatosis syndrome)
Rizzo JF, et al. Optic atrophy in familial dysautonomia. Am J Ophthalmol 1986; 102:463-
467.
Roy FH. Ocular syndromes and systemic diseases, 3rd 00. Philadelphia: Lippincott
OPTIC-NERVE HYPOPLASIA
A. Down syndrome (trisomy 21)
B. Deletion of long chromosome (13)
C. Edward syndrome (trisomy 18)
D. Patau syndrome (trisomy 13)
E. Ring chromosome mosaicism
F. 13 q deletion syndrome
2. Idiopathic
3. Neurologic conditions
A. Agenesis of the corpus callosum
B. Anencephaly
C. Basal encephalocele
D. Behavioral problems
E. Cerebellar atrophy
F. Cerebral atrophy
G. Cerebral infarcts
H. Cerebral palsy
I. Colpocephaly
J. Congenital suprasellar tumors
K. Congenital third, fourth, and sixth nerve palsies and up-gaze palsies
L. Encephaloceles
M. Hydranencephaly
N. Hydrocephaly
O. Mental retardation
P. Migration disturbances
Q. Perinatal encephalopathy
R. Porencephaly
S. Posterior pituitary ectopia
4. Ocular conditions
A. Albinism
B. Aniridia
C. Astigmatism
D. Blepharophimosis
E. Colobomas (optic disc and chorioretinal)
F. High myopia
G. Microphthalmos
H. Retinal vascular tortuosity
5. Systemic conditions
A. Aicardi syndrome
B. Albinism
C. Chondrodysplasia punctata
D. Cleft lip and palate
E. Diabetes mellitus (maternal)-segmental optic nerve hypoplasia in infant
F. Duane retraction syndrome
G. Fetal alcohol syndrome, especially pituitary abnormalities with isolated
tortuosity of retinal veins
H. Goldenhar-Gorlin syndrome
I. Hemifacial atrophy
J. Hypertelorism
K. Intrauterine infections-including cytomegalovirus and hepatitis
(1) Cytomegalovirus
(2) Hepatitis
L. Inherited (autosomal dominant or recessive)
M. Klippel-Trenaunay-Weber syndrome
N. Meckel syndrome
O. Median cleft face syndrome
P. de Morsier syndrome (septooptic dysplasia and mutations in the homeobox
gene HESX1/hesxl)
Q. Osteogenesis imperfecta
R. Potter syndrome
S. Syndrome of nevus sebaceus of Jadassohn
T. Topless optic disk syndrome
6. Toxins (maternal use of)
A. Alcohol
B. Lysergic acid diethylamide (LSD)
C. Phencyclidine (PCP)
D. Phenytoin
E. Quinine
F. Tobacco
Brodsky MC, et al. Congenital optic disc anomalies [review]. Surv Ophthalmol 1994;
32:89-112.
Brodsky MC. Optic nerve hypoplasia with posterior pituary ectopia: male predominance
and nonassociation with breech delivery. Am J Ophthalmol 1999; 127, 2:238-239.
Helstrom A, et al. Optic nerve hypoplasia with isolated tortuosity of the retinal veins. Arc
Ophthalmol 1999; 117: 880-884.
Chismire KJ, Witkop GS. Angle dysgenesis in a patient with blepharophimosis

Siatkowski RM, et al. The clinical, neuroradiographic, and endocrinologic profile of

patients with bilateral optic nerve hypoplasia. Ophthalmology 1997; 104:493-496.
OPTIC NEURITIS (PAPILLITIS AND RETROBULBAR NEURITIS

This condition is characterized by progressive loss of vision and possibly
complete amaurosis; pain in or behind the eye, especially on lateral movement; Marcus
Gunn pupillary phenomenon; and central or paracentral scotoma.
1. Demyelinating and degenerative diseases

A. Adrenoleukodystrophy
B. Hereditary ataxia (Brown-Marie syndrome)
C. Multiple sclerosis
D. Opticomyelitis (Devic disease)
2. Drugs, poisons, vaccines
A. Drugs, including the following:
acetohexamide chloral hydrate (?) diphtheria and tetanus
acetyldigitoxin chloramphenicol toxoids (adsorbed)
alcohol chloroprocaine (?) diphtheria and tetanus
allobarbital chlorpropamide (?) toxoids and pertussis
aminosalicylate cholecalciferol diphtheria toxoid
aminosalicylic acid (?) cisplatin (adsorbed)
amiodarone (?) clindamycin disulfiram
amitriptyline clophene (?) ergocalciferol (?)
amobarbital cyclobarbital ergonovine
aprobarbital cyclopentobarbital ergotamine
barbital cycloserine (?) ethambutol
bromisovalum deferoxamine ethchlorvynol
broxyquinoline desipramine ethionamide
bupivacaine (?) deslanoside etidocaine (?)
butabarbital dextrothyroxine (?) etoposide
butalbital didanosine etretinate
butallylonal diethylpropion (?) fenoprofen
butethal digitalis ferrocholinate (7)
calcitriol (?) digitoxin ferrous fumarate (7)
caramiphen digoxin ferrous gluconate (7)
carbromal diiodohydroxyquin ferrous succinate
carmustine ferrous sulfate (7)
fluorouracil (7) methysergide smallpox vaccine
gitalin metronidazole(?) streptomycin
glyburide minoxidil(?) sulfacetamide
heptabarbital mumps virus vaccine sulfachlorpyridazine
hexethal (live) sulfacytine
hexobarbital naproxen sulfadiazine
ibuprofen nialamide (?) sulfadimethoxine
imipramine nitrofurantoin (?) sulfamerazine
indomethacin (?) nortriptyline sulfameter
influenza virus vaccine nystatin sulfamethizole
interferon alpha, beta, or oral contraceptives sulfamethoxazole
gamma ouabain sulfamethoxypyridazine
iodide and iodine oxyphenbutazone sulfanilamide
solutions and penicillamine sulfaphenazole
compounds pentobarbital sulfapyridine
iodochlorhydroxyquin phenobarbital sulfasalazine
iodoquinol phenylbutazone sulfathiazole
iron dextran (?) piroxicam (?) sulfisoxazole
iron sorbitex (?) poliovirus vaccine (?) sulindac (?)
isocarboxazid (?) polysaccharide-iron talbutal
isoniazid complex (?) tamoxifen (?)
isotretinoin prilocaine (?) tetanus immune globulin
kanamycin (?) primidone (?)
lanatoside C probarbital tetanus toxoid (?)
levothyroxine (?) procaine (?) thiamylal
lidocaine procarbazine thiopental
liothyronine (?) propoxycaine (7) thyroglobulin (?)
liotrix (?) protripty line thyroid (?)
measles and rubella quinacrine tolazamide
virus vaccine (live) quinidine tolbutamide
measles, mumps, and rabies immune globulin trichloroethylene
rubella virus vaccine rabies vaccine tryparsamide
mephobarbital radioactive iodides vaccine (adsorbed)
mepivacaine (?) rifampin (?) vinbarbital
metharbital rubella and mumps virus vinblastine
methitural vaccine (live) vincristine
methohexital rubella virus vaccine vitamin D
methyl alcohol (live) vitamin D2 (?)
methylergonovine secobarbital vitamin D3 (?)
B. Poisons (inhalation, skin absorption, or ingestion): alcohol, arsenicals (inorganic,

gaseous, or organic), carbon disulfide, carbon tetrachloride, chlorodinitrobenzene and
dinitrobenzene, copper, dinitrotoluene, Lysol solution, mercury, methyl bromide, methyl
alcohol, siderosis (exogenous: intraocular foreign body or endogenous: iron metabolism
disorders), tobacco, toluene (methyl benzene), trichlorethylene, tricresil phosphate,
venoms (e.g., bee sting), vinyl benzene (styrene)
C. Vaccines and toxoids: Bacille Calmette-Guérin (bCG) vaccination, diphtheria toxoid
(absorbed), diphtheria and tetanus toxoids (absorbed), influenza virus vaccine, measles or
mumps or rubella live vaccine, poliovirus vaccine, rabies immune globulin, rabies
vaccine, smallpox vaccine, tetanus immune globulin (?), tetanus toxoid (?), bee and wasp
sting.
3. Infection and inflammation
A. Bacterial
(1) Anthrax
(2) Botulism (toxin from clostridium botulinum)
(3) Brucellosis (undulant fever)
(4) Diphtheria
(5) Endocarditis
(6) Leptospirosis (Weil syndrome)
(7) Lyme disease (borreliosis, relapsing fever)
(8) Mycoplasma pneumoniae
(10) Streptococcus (scarlet fever)
(12) Tuberculosis
(13) Typhoid fever (abdominal typhus)
B. Fungal
(1) Candidiasis
(3) Mucormycosis
(4) Torulosis (cryptococcus)
C. Viral
(1) Acquired immune deficiency syndrome (AIDS)
(2) Bornholm disease (epidemic pleurodynia)
(3) Chickenpox (varicella)
(4) Epidemic keratoconjunctivitis
(5) Equine encephalitis
(6) Hepatitis A, B, C
(8) Influenza
(9) Measles (rubeola)
(10) Mumps
(11) Pappataci fever (sandfly fever)
(12) Poliomyelitis
(13) Smallpox
(14) Yellow fever
D. Protozoan
(1) Malaria
(2) Toxoplasmosis
(3) Trypanosomiasis
E. Rickettsia
(1) Boutonneuse fever rickettsia (Marseilles fever)
(2) Japanese river fever (typhus)
(3) Q fever
(4) Rocky Mountain spotted fever
F. Orbit
(1) Herpes zoster
(2) Infections of the gasserian ganglion
(3) von Mikulicz-Radecki syndrome (dacryosialoadenopathy)
(4) Rollet syndrome (orbital apex syndrome)
(5) Tolosa-Hunt syndrome (painful ophthalmoplegia)
G. Helminth infestations
(1) Acanthamoeba
(2) Echinococcosis (hydatid cyst)
(3) Onchocerciasis (river blindness)
(4) Toxocariasis (nematode ophthalmia syndrome)
(5) Trichinellosis
H. Spread from sphenoid and posterior ethmoidal sinuses
I. Postinfectious
(2) Reye syndrome (acute encephalopathy syndrome)
(3) Subacute sclerosing panencephalitis (Dawson disease)
(4) Vogt-Koyanagi-Harada syndrome (uveitis-vitiligo-alopecia-poliosis
syndrome)
4. Noninfectious arteritis, hypersensitivity vasculitis
A. Involving small vessels
(1) Drugs
(2) Henoch-Schönlein
B. Involving small and medium-sized vessels
(2) Necrotizing granulomatous arthritis
a. Sarcoidosis
b. Wegener granulomatosis (Wegener syndrome)
(3) Buerger disease (thromboangiitis obliterans)
(4) Localized arteritis
a. Idiopathic
b. Polyarteritis nodosa
C. Involving large, medium and small vessels
(1) Arteritis in collagen vascular disease
a. Behçet disease (oculobuccogenital syndrome)
b. Progressive systemic sclerosis (PSS; scleroderma)
c. Rheumatoid arthritis
d. SLE
(2) Giant cell (temporal) arteritis
(3) Takayasu syndrome (aortic arch syndrome)
D. Idiopathic paroxysmal digital cyanosis (Raynaud disease)
5. Others
A. Chorioretinitis
B. Cystic fibrosis syndrome
C. Hutchinson-Gilfor (progeria) syndrome
D. Hysteria
E. McCune-Albright syndrome (fibrous dysplasia)
F. Naegeli syndrome (melanophoric nevus)
G. Paget disease (osteitis deformans)
H. Parkinson syndrome (paralysis agitans)
I. Relapsing polychondritis
J. Stevens-Johnson syndrome (erythema multiforme exudativum)
K. Uveitis, including sympathetic ophthalmia
A. Endocrine
(3) Hyperthyroidism (Basedow syndrome)
(4) Hyperthyroidism
(5) Juvenile diabetes-dwarfism-obesity syndrome (Mauriac syndrome)
(6) Lactation
(7) Pregnancy
(8) Puberty
(9) Retinohypophysary syndrome (Lijo Pavia-Lis syndrome)
B. Nutritional diseases
(1) Beriberi (vitamin B deficiency)
(2) Carcinomatosis
(3) Hyperemesis gravidarum
(4) Pellagra (vitamin B deficiency)
C. Rheumatic disease, arthritis
(1) Felty syndrome
(2) Juvenile rheumatoid arthritis (Still disease)
(3) Polymyalgia rheumatica
(4) Reiter syndrome (polyarthritis enterica)
D. Miscellaneous
(1) Amyloidosis (Lubarsch-Pick syndrome)
(2) Chronic glomerulonephritis with secondary renal hypertension or
pyelonephritis
(3) Emphysema
(4) Hepatic failure
(5) Hypertension
(6) Porphyria
(7) Trauma
A. Mechanical
B. Radiation
(1) Electromagnetic
a. High voltage/lighting
b. Microwave
c. Laser bum
d. X-ray
(2) Radioactive source
a. a-ruthenium
b. b-betatron
c. g-cobalt
d. pisotope
8. Tumors
A. Craniopharyngioma
B. Hemangiopericytoma of optic nerve
C. Myeloproliferative diseases
(1) Hodgkin disease
(2) Leukemia
(3) Lymphoma
D. Neuroblastoma
Beck RW, et al. Fellow eye abnormalities in acute unilateral optic neuritis.
Woburn, MA: Butterworth-Heinemann, 200 l.
Maltzman JS, et al. Optic neuropathy occurring after bee and wasp sting. Ophthalmology
2000; 107:193-195.
Straussberg R, et al. Epstein-Barr virus infection associated with encephalitis and optic
neuritis. J Pediatr Ophthalmol Strabismus 1993; 30:262-263.
Extracted Table (Several unreadable titles)
PSEUDOOPTIC NEURITIS (LESIONS THAT MIMIC OPTIC NEURITIS)
1. Congenital retinoschisis
2. Hematoma
5. Retinal lesions that also exhibit metamorphopsia, e.g., serous or angiospastic
retinopathy
6. Tumors
A. Disc
(1) Gliomas
(2) Meningiomas
(4) Neurofibromas
B. Expanding lesions of anterior and middle cranial fossa producing central
scotoma
(1) Craniopharyngiomas
(2) Ectopic pinealomas
(3) Meningiomas
(4) Metastatic carcinomas
(5) Myeloproliferative diseases
a. Hodgkin disease
b. Lymphomas
c. Plasmocytoma
(6) Nasopharyngeal carcinomas
(7) Pituitary adenomas
OPTICOCILIARY SHUNTS (TORTUOUS, ECTATIC CHANNELS FROM

OPTIC
NERVE TO CHOROID)
1. Arachnoid cyst of the optic nerve

2. Central retinal vein occlusion (see p. 468-472)
3. Chronic atrophic papilledema
4. Drusen of the optic nerve
5. Optic-nerve glioma
6. Primary nerve sheath meningioma
7. Sickle cell trait
8. Sphenoorbital meningioma
Dowhan TP, et al. Optociliary shunts and sickle retinopathy in a woman with sickle cell
trait. Ann Ophthalmol 1990; 22:66-69.
Mendoza RM, et al. Optociliary veins in optic nerve sheath meningioma. Ophthalmology
1999; 106:311-318.
PAPILLEDEMA (SWELLING OF OPTIC DISC)
1. Drugs, poisons, and vaccines

A. Drugs (those in all capitalized letters are drugs that also cause pseudotumor
cerebri):
acetophenazine amiodarone antimony potassium
ADRENAL CORTEX amobarbital tartrate
INJECTION AMPHOTERICIN B antimony sodium
ALDOSTERONE antimony lithium tartrate
allobarbital thiomalate
antimony sodium etoposide metharbital
thioglycollate FLUDROCORTISONE methdilazine
aprobarbital fluorometholone methitural
aspirin fluphenazine methohexital
auranofin (?) FLUPREDNISOLONE methotrimeprazine
aurothioglucose (?) GENTAMICIN methyl alcohol
aurothioglycanide (?) glutethimide methylene blue
Azathioprine gold Au 198 METHYLPREDNISOLONE
barbital gold sodium methyprylon
benzathine penicillin G thiomalate (?) mitotane
bromide (?) gold sodium nadolol (?)
bupivacaine (?) thiosulfate (?) NALIDIXIC ACID
butabarbital heptabarbital Naproxen (?)
butalbital HEXACHLOROPHENE NITROFURANTOIN
butallylonal hexethal NITROGLYCERIN
butaperazine hexobarbital NORPLANT
butethal hydrabamine ofloxacin
calcitriol phenoxymethyl ORAL
carbamazepine penicillin CONTRACEPTIVES
carbon dioxide HYDROCORTISONE OXYTETRACYCLINE
carphenazine IBUPROFEN PARAMETHASONE
cephaloridine (?) INDOMETHACIN penicillamine
chlorambucil INSULIN-LIKE pentobarbital
chloramphenicol (?) GROWTH perazine
chloroprocaine (?) FACTOR I PERHEXILINE
chlorpromazine interferon pericyazine
CHLORTETRACYCLINE interferon alpha, beta, or perphenazine
cholecalciferol gamma phenelzine (?)
cisplatin isocarboxazid (?) phenobarbital
colchicine isoniazid phenoxymethyl
CORTISONE ISOTRETINOIN penicillin
cyclobarbital KETOPROFEN PHENYLPROPANOL-
cyclopentobarbital LEVODOPA AMINE
DANAZOL LEVOTHYROXINE PHENYTOIN
DEMECLOCYCLINE lidocaine piperacetazine
DESOXYCORTICO- LIOTHYRONINE POTASSIUM
STERONE LITHIUM PENICILLIN G
DEXAMETHASONE CARBONATE POTASSIUM
DEXTROTHYROXINE LITHIUM CITRATE PENICILLIN V
didanosine MANGANESE POTASSIUM
diethazine mephobarbital PHENETHICILLIN
DOXYCYCLINE mepivacaine (?) PREDNISOLONE
ELTROXIN MEPREDNISONE PREDNISONE
ethambutol mesoridazine prilocaine (?)
ethopropazine METHACYCLINE primidone
etidocaine (?) methaqualone (?) probarbital
procaine (?) sulfachlorpyridazine thiethylperazine
PROCAINE sulfacytine thiopental
PENICILLIN G sulfadimethoxine thiopropazate
procarbazine sulfamerazine thioproperazine
prochlorperazine sulfameter thioridazine
promazine sulfamethazine THYROGLOBULIN
promethazine sulfamethizole THYROID
propiomazine sulfamethoxazole tranylcypromine (?)
propoxycaine (?) sulfamethoxypyridazine trifluoperazine
PYRIDOXINE sulfanilamide triflupromazine
quinine sulfaphenazole Trimeprazine
RETINOIDS sulfapyridine vinbarbital
secobarbital sulfasalazine VITAMIN A
sodium sulfathiazole (RETINOL)
antimonylgluconate sulfisoxazole vitamin D (calcitriol)
sodium salicylate sulthiame vitamin D2
stibocaptate talbutal (ergocalciferol)
stibogluconate tamoxifen vitamin D3
stibophen TETRACYCLINE (cholecalciferol)
sulfacetamide thiamylal
B. Poisons (inhalation, skin absorption, or ingestion)

(1) Carbon dioxide
(2) Lead
(3) Methyl alcohol
C. Vaccines
(1) Diphtheria-tetanus toxoids-pertussis vaccine (absorbed)
(2) Influenza virus vaccine
(3) Measles or mumps or rubella live vaccine
2. Intracranial causes-usually bilateral
A. Tumors
*(1) Frontal lobe lesion-mental changes (apathy, euphoria, and social
behavioral changes); normal visual field if confined to frontal lobe; most
likely, tumors are medulloblastoma, meningioma, astrocytoma,
glioblastoma, or metastasis from lung or breast
*(2) Temporal lobe lesions-formed hallucinations, superior homonymous
quadrantanopia, or homonymous hemianopia, ipsilateral mydriatic fixed
pupil and oculomotor paresis, and contralateral facial palsy; most likely,
tumors are medulloblastoma, meningioma, astrocytoma, glioblastoma, or
metastasis from lung or breast
*(3) Parietal lobe lesions-visual agnosia such as alexia or dyslexia,
complete homonymous hemianopia, or inferior homonymous
quadrantanopia, disturbances of trigeminal nerve, including decreased
corneal sensation and positive (asymmetric response) optokinetic
nystagmus; most likely caused by:
a. Astrocytoma
b. Glioblastoma
c. Medulloblastoma
d. Meningioma
e. Metastasis from lung or breast
(4) Occipital lobe lesions-unformed visual hallucinations and
homonymous congruous visual field defect; most likely caused by the
following:
a. Astrocytoma
b. Glioblastoma
c. Hemangioma
d. Meningioma
e. Metastasis from lung or breast
(5) Third-ventricle and sellar lesions-visual field of bitemporal hemianopia
or unilateral blindness and contralateral temporal hemianopia; most likely,
tumors are craniopharyngioma
(6) Fourth-ventricle and cerebellum lesions-ataxia, asynergy, dysmetria,
hypotonia, and acquired jerk nystagmus, usually horizontal and more
pronounced in lateral gaze; most likely caused by:
a. Astrocytoma
b. Hemangioblastoma
c. Medulloblastoma
d. Metastasis from lung or breast
7) Cerebellar-pontine angle tumor such as Cushing syndrome II (acoustic
neuroma syndrome)
(8) Base skull tumor, such as Garcin syndrome (half-base syndrome)
(9) Chiasmal tumor such as Fröhlich syndrome (dystrophia
adiposogenitalis)
(10) Neuroblastoma
(11) Russell syndrome (diencephalic syndrome)
(12) Zollinger-Ellison syndrome (polyglandular adenomatosis syndrome)
B. Decreased intracranial capacity, such as in acrocephalosyndactyly (Apert
disease), Arnold-Chiari syndrome (cerebellomedullary malformation syndrome),
craniofacial dysostosis (Crouzon disease), craniostenosis, hypertelorism, and
tower skull (oxycephaly)
C. Pseudotumor cerebri (Symonds syndrome)-bilateral papilledema and increased
intracranial pressure but negative neurologic and general physical findings
(2) Autosomal dominant endosteal hyperostosis
(3) Chronic respiratory insufficiency
(4) Familial Mediterranean fever
(5) Hypertension
(6) Multiple sclerosis
(7) Polyangiitis overlap syndrome
(8) Psittacosis
(9) Renal disease
(10) Reye syndrome
(11) Sarcoidosis
(12) SLE
(13) Thrombocytopenia purpura
(14) Vitamin A (excessive) after overeating carrots in a weight loss
program
(15) Drugs including the following:
(absorbed) levodopa (?) hexachlorophene nitrofurantoin
adrenal cortex injection hydrabamine nitroglycerin
aldosterone penicillin V ofloxacin
amiodarone hydrocortisone oral contraceptives
benzathine penicillin G ibuprofen (?) oxytetracycline
betamethasone indomethacin paramethasone
chlorambucil isotretinoin penicillin G
chlortetracycline ketoprofen penicillin V
cortisone leuprolide acetate perhexiline
danazol levonorgestrel phenylpropanolamine
demeclocycline levothyroxine phenytoin
desoxycorticosterone liothyronine potassium
dexamethasone liotrix potassium phenethicillin
diptheria and tetanus lithium carbonate prednisolone
toxoids and pertussis manganese prednisone
vaccine medroxyprogesterone procaine
doxycycline medrysone tetracycline
etretinate meprednisone thyroglobulin
fludrocortisone methacycline thyroid
fluprednisolone methylprednisolone triamcinolone
gentamicin minocycline vitamin A
griseofulvin nalidixic acid
(16) Frankl-Hochwart syndrome (pineal-neurologic-ophthalmic syndrome)

(17) Glomus jugulare tumor
(18) Iron-deficiency anemia
(19) Menarche
(20) Pregnancy
(21) Thrombosis of the sagittal or lateral sinus, such as that following
otitis media in children
(22) Yersinia pseudotuberculosis
3. Neurologic disorders
A. Cerebral palsy
B. Foster-Kennedy syndrome
(1) Aneurysm of internal carotid, anterior cerebral, or anterior
communicating artery
(2) Arteriosclerotic plaques of internal carotid or anterior cerebral arteries
(3) Chiasmal arachnoiditis secondary to trauma, spinal anesthesia, or
syphilis
(4) Craniopharyngioma with forward extension
(5) Frontal lobe tumors or abscess
(6) Glioma of the intracranial portion of optic nerve
(7) Internal hydrocephalus because of tumor of posterior fossa
(8) Old unilateral optic nerve atrophy (e.g., consecutive ischemic optic
neuropathies)
(9) Olfactory groove, sphenoid ridge and suprasellar meningioma
C. High cerebrospinal fluid protein content and defective absorption (e.g.,
Guillain-Barré syndrome (Acute Infectious Neuritis))
D. Muscular dystrophy
E. Parkinson syndrome (shaking palsy)
F. Status dysraphicus syndrome (Passow syndrome, syringomyelia)
G. Subdural or subarachnoid hemorrhage
4. Miscellaneous
A. Abscess
B. Angioedema
C. Brown-Séquard syndrome
D. Camurati-Engelmann syndrome (progressive diaphyseal dysplasia)
E. Chediak-Higashi syndrome (anomalous leukocytic inclusions with
constitutional stigma)
F. Churg-Strauss syndrome (allergic granulomatosis and angiitis)
G. Citrullinemia (late onset)
H. Degos syndrome (malignant atrophic papulosis)
I. Fabry disease (angiokeratoma corporis diffusum)
J. Hydrocephalus
K. Kenny syndrome
L. McCune-Albright syndrome (fibrosus dysplasia)
M. Nocturnal hypoventilation
N. Pelizaeus-Merzbacher syndrome (aplasia axialis extracorticalis congenita)
O. Polymyalgia rheumatica
P. Primary hyperoxaluria type
Q. Renal insufficiency
5. Ocular cause-usually unilateral
A. Acute glaucoma
B. ACME (Irvine-Gass syndrome)
C. Central retinal vein or artery occlusion
D. Hypotony, including that following intraocular surgery
E. Inflammatory
(1) Bird-shot retinochoroidenopathy
(2) Gumma of nerve head
(3) Juxtapapillary choroiditis
(4) Neuroretinitis (see p. 563)
(5) Retinal vasculitis
(6) Rocky Mountain spotted fever
(7) Sarcoidosis
(8) Tuberculoma of nerve head
(9) Uveitis
(10) Vasculitis
F. Trauma
G. Tumors
(1) Glioma
(2) Hemangioma
(3) Melanocytoma
(4) Melanotic sarcoma
(5) Neurofibromatosis (von Recklinghausen disease)
(6) Periocular and ocular metastatic tumors
(7) Secondary carcinoma
(8) Tuberous sclerosis
6. Orbital cause-usually unilateral, may have exophthalmos
A. Aneurysm of the ophthalmic artery
B. Orbital abscess
C. Rollet syndrome (orbital apex syndrome)
D. Scaphocephaly syndrome (craniofacial dysostosis)
E. Sinusitis
F. Superior orbital fissure syndrome (Rochon-Duvigneaud syndrome)
G. Trauma
H. Tumors
(1) Benign
a. Cystic adenoma
b. Dermoid cyst
c. Osteopetrosis (Albers-Schonberg disease)
d. Paget disease
(2) Malignant
a. Fibrosarcoma
b. Glioma
c. Hutchinson-Pepper syndrome
d. Lacrimal gland
e. Lymphosarcoma
f. Myosarcoma
g. Osteosarcoma
h. Secondary metastasis and extension from nasopharynx or
sinuses
(3) Orbital invasion by intracranial tumor (e.g., chordoma)
7. Systemic diseases-usually bilateral
A. Blood dyscrasias
(1) Iron-deficiency anemias
(3) Thrombocytopenic purpura
B. Carbohydrate metabolisms disorders
(2) ML III
(3) MPS II (Hunter syndrome)
(4) MPS VI (Maroteaux-Lamy syndrome)
C. Cardiopulmonary insufficiency
(1) Chronic bronchitis
(2) Congenital heart disease
(3) Cystic fibrosis of lungs
(4) Pickwickian syndrome
(5) Pulmonary emphysema
D. Collagen diseases
(1) Polyarteritis nodosa
(2) PSS (scleroderma)
(4) SLE
E. Endocrine
(2) Diabetes mellitus (Willis disease)
(3) Hyperparathyroidism
(4) Hyperthyroidism (Basedow syndrome)
(5) Hypothyroidism
(6) Hypocalcemia
(8) Hypophosphatasia
(9) Idiopathic hypercalcemia (Drummond syndrome)
(10) Menses
(11) Pituitary deficiency
(12) Pregnancy
(13) Pseudohypoparathyroidism syndrome
(14) Suppression of adrenal function from prolonged use of steroids
(15) Suprarenal-sympathetic syndrome
F. Giant cell (temporal arteritis)
G. Hypertension/arteriosclerosis
H. Infectious (rare usually optic neuritis)
(1) AIDS
(2) Anterior poliomyelitis
(3) Bang disease (brucellosis)
(4) Chickenpox
(6) Echinococcosis (hydatid cyst)
(7) Encephalitis
(9) Lyme disease (borreliosis, relapsing fever)
(10) Malaria
(11) Meningitis
(12) Mycoplasma pneumoniae
(13) Parasitic infections (e.g., cysticercosis, cryptococcus)
(14) Parinaud syndrome (divergence paralysis)
(16) Presumed ocular histoplasmosis
(17) Psittacosis
(18) Sandfly fever (Pappataci fever)
(19) Trichinellosis
(20) Whipple disease (intestinal lipodystrophy)
I. Postinfectious
(2) Reye syndrome (acute encephalopathy syndrome)
(3) Subacute sclerosing panencephalitis (Dawson disease)
(4) Vogt - Koyanagi - Harada syndrome (uveitis-vitiligo-alopecia-poliosis)
J. Myeloproliferative diseases
(1) Histiocytosis X (lipoid granuloma)
(2) Hodgkin disease
(3) Leukemia
(4) Multiple myeloma
(5) Mycosis fungoides (Sézary syndrome)
(6) Polycythemia vera
K. Paraproteinemias
(2) Macroglobulinemia
(3) Mediterranean fever
(4) POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy,
M protein, and skin changes)
L. Nutritional diseases
(1) Beriberi (thiamine deficiency)
(2) Pellagra (niacin deficiency)
(3) Plummer-Vinson syndrome (deficiency of vitamin B complex and
iron)
(4) Vitamin B12 deficiency
M. Sarcoidosis (Heerford syndrome, Schaumann syndrome)
8. Trauma
A. Battered/shaken baby syndrome
B. Cerebral hemorrhage
C. Purtscher syndrome
9. Vascular malformations
A. Arteriovenous fistula
B. Aneurysms
(1) Bonnet-Dechaume-Blanc syndrome (neuroretinoangiomatosis)
(2) Foramen lacerum syndrome (aneurysm of internal carotid artery
syndrome)
(3) Superior vena cava syndrome
C. Cavernous sinus thrombosis (Foix syndrome)
Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri.

Opthalmology 1998; 105: 1686-1693.
Donahue SP. Recurrence of idiopathic intracranial hypertension after weight loss. The
carrot craver. Am J Ophthalmol 2000; 130:850-851.
Fraunfelder FT Fraunfelder FW. Drug-induced ocular side effects and drug interactions.
Miller N, Newman N. Walsh & Hoyt's clinical neuro-ophthalmology. Philadelphia:

Lippincott Williams & Wilkins, 1999.
PSEUDOPAPILLEDEMA (MAY BE MISTAKEN FOR SWELLING OF OPTIC

NERVE)
1. Arteriovenous aneurysms (racemose aneurysms) of the retina (Wyburn-Mason

syndrome)
2. Bergmeister papilla
3. Cervicooculoacousticus syndrome
4. Down syndrome
5. Drusen of optic nerve (see p. 559)
6. Epipapillary membrane and Bergmeister papilla
7. Fuchs coloboma (partial)
8. Hematoma
9. High hyperopia or astigmatism
10. Juvenile diabetes mellitus (Mauriac syndrome)
11. Medullated nerve fibers (opaque nerve fibers)
12. Normal variant
13. Opacities or haziness of the media, especially nuclear sclerosis of the lens
14. Optic neuritis or papillitis (see p. 578)
15. Peripapillary retinal hemangioma
16. Sarcoidosis (Schaumann syndrome)
17. Tilted disc (partial)
18. Tortuosity and anomalous early branching of the retinal vessels
19. Tumors of disc
A. Gliomas
B. Meningiomas
C. Metastatic
D. Neurinoma
E. Neurofibroma
Catalano RA, Simon JW. Optic disk elevation in Down's syndrome. Am J Ophthalmol
1990; 110:28-32.
Mosby, 1972.
Extracted Table Unilateral papilledema
Extracted Table Bilateral papilledema
PERIPAPILLARY SUBRETINAL NEOVASCULARIZATION
1. Excessive laser treatment

2. Optic disc drusen
3. Optic nerve coloboma
4. Presumed histoplasmosis syndrome
5. Presumed sarcoidosis
6. Serpiginous peripapillary choroiditis
Gragoudas ES, Regan CDJ. Peripapillary subretinal neovascularization in presumed

sarcoidosis. Arch Ophthalmol 1981; 99: 1194.
Yedavally S, Frank RN. Peripapillary subretinal neovascularlzation associated with

coloboma of the optic nerve. Arch Ophthalmol 1993; 111:552-553.
PIGMENTED TUMORS OF OPTIC DISC
1. Drusen
3. Hemangioma of the disc with hemorrhages and secondary pigmentation
5. Melanocytomas
6. Metastases
Apple DJ, et al. Congenital anomalies of the optic disc. Surv Ophthalmol 1982; 27:3.
PSEUDOGLAUCOMATUS ATROPHY OF OPTIC DISC

This condition involves cupping of the nerve head with optic atrophy and field
defects simulating true glaucoma but without ocular hypertension.
1. Arteriosclerosis
2. Congenital anomalies of the optic disc
A. Branching of vessels behind the lamina so that the individual branches appear
at the disc margins
B. Coloboma within the nerve sheath
C. Congenital coloboma of the optic disc
D. Morning-glory anomaly
E. Oblique insertion of the optic nerve
F. Traction of the disc with bowing of the scleral crescent
3. Giant cell (temporal) arteritis
4. Optic pit
5. Patients using digitalis
6. Reduced blood supply to optic nerve (e.g., acute hypotension, blood loss [severe]
carotid insufficiency, gastrointestinal bleeding, ischemic optic neuropathy, myocardial
infarction, pernicious anemia)
7. Schnobel cavernous atrophy
8. Syphilitic optic-nerve atrophy
9. Tumors arising near the chiasm
Jonas JB, et al. Pseudoglaucomatous physiologic large cups. Am J Ophthalmol 1989;

107:137-144.
Kolker AE, Hetberington J. Becker-Schaffer's diagnosis and therapy of the glaucomas,

6tb ed. St. Louis: CV Mosby, 1989.
Moore M, et al, Progressive optic nerve cupping and neural rim decrease in a patient with
bilateral autosomal dominant optic nerve colobomas. Am J Opthalmol 2000; 129:517-
520.
Vaughan D, et al. General ophthalmology, 12tb ed. Norwalk, CT: Appleton & Lange,
1989.
TEMPORALL Y DISPLACED DISC (DRAGGED DISC)
1. Abnormal tortuous retinal vessels temporally

2. Ectopic macula
3. ROP
4. Temporally displaced vessels
Gow J, Oliver GL. Familial exudative vitreoretinopathy. Arch Ophthalmol 1971; 86:150-
155.
18
Visual-Field Defects
CONTENTS
Pseudo-visual-field defects 604
Bilateral central scotomas 605
Enlargement of blind spot 607
Arcuate (cuneate) scotoma 608
Unilateral sector-shaped defects 608
Peripheral-field contraction 608
Altitudinal hemianopia 610
Binasal hemianopia 610
Bitemporal hemianopia 611
Homonymous quadrantanopia 612
Crossed quadrantanopia 613
Homonymous hemianopia 613
Spiral-field defects 614
Double homonymous hemianopia 614
PSEUDO- VISUAL-FIELD DEFECTS
1. Facial contour
A. Prominent nose
B. Bushy, projecting eyebrows
C. High cheekbones
D. Ptosis or blepharochalasis
E. Sunken globes
F. Fracture of orbit
2. Corneal opacities
3. Lenticular opacities, especially if miotics are used, will depress fields and exaggerate
existing scotomas
4. Aphakia without lens or with convex lens; little distortion with contact lens or
intraocular lens
5. Dull patient; patient may be mentally defective, have toxemia, arteriosclerosis, cerebral
tumor, brain abscess, or increased intracranial pressure
6. Pupillary size
A. Decrease in miotic field, especially with opacities of ocular media
7. Uncorrected refractive errors-correct for distance testing
8. Head tilting when the head is tilted toward the left shoulder; the right blind spot is
elevated; when the head is tilted toward the right shoulder, the right blind spot is lowered
9. Environmental artifacts
A. Reduction in illumination of screen and test objects magnifies field defect
B. Variation in size of test object changes field defect
C. Standard distance of patient from screen
D. Attention of patient
E. Technique of examiner
10. Psychologic artifacts
A. Patient's misunderstanding of test
B. Tiring of patient by prolonged testing
C. Malingering-isopters at different distances are inconsistent
D. Hysteria-spiral field defects may be found
11. Frames of glasses and segments of multifocal lenses
12. Colored contact lenses
Insler MS, et al. Visual field constriction caused by colored contact lenses. Arch
Ophthalmol 1988; 106:1680-1682.
Meyer DR, et al. Evaluating the visual field effects of blepharoptosis using automated
static perimetry. Ophthalmology 1993; 100:651-659.
BILATERAL CENTRAL SCOTOMAS

These are bilateral macular defects with decreased visual acuity; scotomas may be
central or centrocecal.
1. Bilateral macular lesions, such as cysts or those due to hemorrhage, edema,

degeneration, detachment, hole, or infection (see p. 439)
2. Bilateral optic-nerve lesions
A. Papilledema with macular edema (see p. 593)
B. Bipituitary adenoma compressing the prechiasmatic segment of the distal optic
nerve
C. Papillitis (see p. 578)
D. Retrobulbar neuritis (see p. 578)
4. Familial optic atrophies (see p. 564)
5. Hyperbaric oxygen
6. Migraine-forerunner of visual aurae
*7. Nutritional deficiency, such as thiamine or vitamin B12 deficiency
8. Pernicious anemia
9. Occipital cortex lesions
10. Toxic agents
A. Aromatic aminocompounds and nitrocompounds-aniline, nitrobenzene,
trinitrotoluene
B. Carbon disulfide
C. Drugs, including:
acetophenazine allobarbital antazoline
acetyldigitoxin aluminum nicotinate (?) aprobarbital
adrenal cortex injection aminosalicylic acid (?) aspirin
alcohol amiodarone barbital
aldosterone amobarbital beclomethasone
alkavervir amodiaquine betamethasone
bromide dimethindene methitural
bromisovalum diphenhydramine methohexital
brompheniramine diphenylpyraline methotrimeprazine
butabarbital diphtheria and tetanus methoxsalen
butalbital toxoids absorbed methyldopa
butallylonal disulfiram methylergonovine
butaperazine doxylamine methylprednisolone
butethal emetine methysergide
caramiphen (?) epinephrine morphine (?)
carbinoxamine ergonovine naproxen
carbon dioxide ergot niacinamide (?)
carbromal ergotamine nicotinic acid (?)
carisoprodol ethambutol nicotinyl alcohol (?)
carphenazine ethchlorvynol ofloxacin
chloramphenicol ethopropazine opium
chloroquine fludrocortisone oral contraceptives
chlorpheniramine fluorometholone ouabain
chlorpromazine fluphenazine oxygen
chlorpropamide (?) fluprednisolone oxyphenbutazone
chlortetracycline gitalin paramethadione
ciprofloxacin heptabarbital paramethasone
cisplatin hexamethonium pentobarbital
clemastine hexethal perazine
clomiphene hexobarbital pericyazine
cobalt (?) hydrocortisone perphenazine
contraceptives hydroxychloroquine phenobarbital
cortisone ibuprofen piperacetazine
cyclobarbital indomethacin (?) prednisolone
danazol influenza virus vaccine prednisone
dapiprazole interleukin 2, 3. and 6 primidone
hydrochloride iodide and iodine probarbital
deferoxamine solutions and prochlorperazine
demeclocycline compounds promazine
deslanoside iothalamic acid promethazine
desoxycorticosterone isoniazid propiomazine
dexamethasone lanatoside C quinacrine
dexbrompheniramine lidocaine quinidine
dexchlorpheniramine lithium carbonate quinine
diatrizoate meglumine medrysone radioactive iodides
and sodium mephobarbital secobarbital
diazoxide meprednisone sodium
diethazine meprobamate sodium salicylate
diethylcarbamazine mesoridazine streptomycin
digitalis methacycline sulfacetamide
digitoxin metharbital sulfachlorpyridazine
digoxin methdilazine sulfadiazine
sulfadimethoxine sulfisoxazole thyroid (?)
sulfamerazine talbutal triamcinolone
sulfameter thiamylal trichloroethylene
sulfamethizole thiethylperazine trifluoperazine
sulfamethoxazole thiopental triflupromazine
sulfamethoxypyridazine thiopropazate trimeprazine
sulfanilamide thioproperazine trimethadione
sulfaphenazole thioridazine vinbarbital
D. Ethyl alcohol
E. Halogenated hydrocarbons-methyl chloride, methyl bromide, iodoform,
trichloroethylene
F. Metals-lead, thallium (inorganic), arsenic
G. Methyl alcohol
H. Tobacco
Harrington DO, Drake MV. The visual fields: text and atlas of clinical perimetry, 6th ed.
St. Louis: CV Mosby. 1990.
Karanjia N, Dacobson DM. Compression of the prechiasmatic optic nerve produces a

junctional scotoma. Am J Ophthalmol 1999; 128, 2:256-258.
ENLARGEMENT OF BLIND SPOT
1. Blind spot syndrome (multiple evanescent white-dot syndrome [MEWS])

2. Coloboma of the optic nerve
adrenal cortex injection ergot oxytetracycline
aldosterone fludrocortisone paramethasone
betamethasone fluorometholone prednisolone
carbon dioxide fluprednisolone prednisone
chlortetracycline hydrocortisone quinacrine
cortisone indomethacin (?) tetracycline
demeclocycline medrysone triamcinolone
desoxycorticosterone methacycline trichloroethylene
dexamethasone methylprednisolone vigabatrin
doxycycline minocycline vitamin A
3. Drusen of the optic nerve (see p. 559)

4. Glaucoma
5. Inferior conus
6. Inverted disc or nasally directed scleral canal
7. Juxtapapillary choroiditis
8. Medullated nerve fibers
9. Multifocal choroiditis
10. Multifocal evanescent white-dot syndrome
*11. Papilledema (pseudotumor cerebri) (see p. 593)
12. Papillitis (see p. 578)
13. Progressive myopia with a temporal crescent
14. Senility-senile halo
15. High-resistance instrument players
Poinoosawmy D, et al. Frequency of asymmetnc visual field defects in normal tension

and high tension glaucoma. Ophthalmology 1998; 105:988-991.
Rebolleda G, et al. Screening of patients taking Vigabatrin. Ophthalmology 2000;
107:1219-1220.
Singh K, et al. Acute idiopathic blind spot enlargement. Ophthalmology 1991; 98:497-
502.
ARCUATE (CUNEATE) SCOTOMA

In this condition, scotoma follows the lines of the nerve fibers in the retina with
the narrow end at the blind spot and broad end at horizontal raphe.
1. Acute bleeding episode

2. Branch artery occlusion
3. Branch vein occlusion
4. Chorioretinitis juxtapapillaris
5. Coloboma of the disc
*7. Glaucoma
8. High myopia
9. Inferior conus
11. Myelinated nerve fibers
Greve EL, Verriest G. Fourth International Visual Field Symposium. The Hague,
Netherlands, 1981.
UNILATERAL SECTOR-SHAPED DEFECTS

In this condition, the narrow end of scotoma characteristically touches the
physiologic blind spot.
1. Optic disc involvement
A. Glaucoma (early stages primarily on nasal side)
B. Papillitis
C. Secondary optic atrophy after choked disc (more on nasal side)
2. Retina
A. Branch artery occlusion (see p. 468-472)
B. Juxtapapillary chorioretinitis
3. Optic nerve-between disc and chiasm
A. Aneurysm
B. Drusen
C. Tumor
Hart WM, Becker B. The onset and evolution of glaucomatous visual field defects.
Spekreijse H, Apkarian PA. Visual pathways. The Hague, Netherlands: Dr. Junk, 1981.
PERIPHERAL-FIELD CONTRACTION
Central vision is present; the patient may complain of poor night vision.
1. Choroiditis-periphery of fundus
*2. Chronic atrophic papilledema (pseudotumor cerebri)
3. Bilateral homonymous hemianopia (if the macular sparing in one homonymous
heminanopia is larger than that in the other homonymous hemianopia, the spared central
portion of the field has small vertical steps, above and below fixation, where the two
areas of macular sparing do not quite coincide.)
A. Cortical blindness with damage to occipital lobe and macular recovery
(1) Anoxia
(2) Carbon monoxide poisoning
(3) Cardiac arrest
(4) Cerebral angiography
(5) Exsanguination
(6) Trauma
B. Stroke of infarction of occipital lobe
acetophenazine butalbital chlorpromazine
acridine butallylonal chlorpropamide (?)
alcohol butaperazine clomiphene
allobarbital butethal cobalt
amobarbital carbon dioxide cortisone
amodiaquine carbon monoxide cyclobarbital
aprobarbital carbromal deslanoside
arsenic carisoprodol desoxycorticosterone
aspirin carphenazine dexamethasone
barbital chloramphenicol diethazine
bromisovalum chloroquine digitalis
digoxin hexethal methitural
disulfiram hexobarbital methohexital
emetine hydrocortisone methotrimeprazine
epinephrine hydroxychloroquine methylprednisolone
ergot ibuprofen methysergide
ethambutol indomethacin morphine (?)
ethchlorvynol iodide and iodine niacinamide (?)
ethopropazine solutions and nicotinic acid (?)
ethylhydrocupreine compounds nicotinyl alcohol (?)
filax mas isoniazid opium
fludrocortisone lanatoside C oral contraceptives
fluorometholone lithium carbonate ouabain
fluphenazine medrysone oxygen
fluprednisolone mephobarbital paramethadione
gitalin mesoridazine pomas
heptabarbital metharbital
hexamethonium methdilazine
5. Drusen of optic disc

6. Frontal-lobe tumors
7. General apathy in a lackadaisical subject
*8. Glaucoma
9. Hysteria and malingering
10. Many conditions in which night blindness occurs (see p. 656)
11. Optic atrophy (see p. 564)
12. Papillitis (see p. 578)
13. Post vitrectomy with fluid-air exchange
14. Retinitis-periphery of fundus
16. Unilateral concentric constriction, excluding diseased retina or glaucoma, suggests
lesion of optic nerve and chiasm
A. Meningioma of tuberculum sellae, sphenoid ridge, or the olfactory groove
B. Tumor of optic nerve
Grover S, et al. Patterns of visual field progression in patients with retinitis pigmentosa.
Ophthalmology 1998; 105: 1060-1075.
Kokame GT. Visual field defects after vitrectomy with fluid-air exchange. Am J
Ophthalmol 2000; 129:653-654.
ALTITUDINAL HEMIANOPIA
This condition comprises defective vision or blindness in the upper or lower
horizontal half of the visual field. It may be unilateral or bilateral; unilateral field defect
is prechiasmal.
1. Anemia-produces bilateral inferior altitudinal hemianopia

*2. Anterior ischemic optic neuropathy
3. Bilateral branch retinal artery occlusion
4. Fusiform aneurysms (arteriosclerotic or congenital)-may produce inferior altitudinal
hemianopia by pressure against the lateral halves of the optic chiasm or nerve
5. Herpes zoster
6. Lesion that presses the chiasm upward against the superior margin of the optic foramen
7. Occipital lobe lesions
A. Hypoxia
B. Stroke
8. Olfactory groove meningioma extending posteroinferior to compress the intracranial
portion of the optic nerve
9. Optic-nerve lesion
A. Anterior ischemia optic neuropathy
B. Coloboma
C. Glaucoma
D. Optic neuritis
E. Papilledema
*F. Trauma
G. Tumor
10. Sclerotic plaques of internal carotid artery or anterior cerebral arteries-pressure of
plaques on optic nerve results in inferior altitudinal hemianopia
11. Following pars plana vitrectomy
Harrington DO, Drake MV. The visual fields: a text and atlas of clinical perimetry, 6th
ed. St. Louis: CV Mosby, 1990.
Miyashita K, et al. Superior altitudinal hemianopia and herpes zoster. Ann Ophthalmol
1993; 25:20-23.
BINASAL HEMIANOPIA
This condition comprises defects in the nasal half of visual fields, usually
incomplete. This condition is due to lateral involvement of chiasm; it presupposes
bilateral lesions.
1. Bilateral occipital lesion (thrombosis)

2. Chiasmic arachnoiditis, postneuritic optic atrophy, and bilateral retrobulbar neuritis of
multiple sclerosis
3. Damage to chiasm
4. Drusen of optic nerve (see p. 559)
5. Fusiform aneurysms-arteriosclerotic or congenital-of internal carotid artery
6. Glaucoma
7. Meningiomas, especially from the lesser wing of the sphenoid bone
8. Nasal quadrant peripheral depression of glaucoma-bilateral and reasonably
symmetrical
9. Pituitary tumor with third ventricle dilatation pushing laterally
10. Retinal damage
11. Severe exsanguination
12. Sclerotic plaques of internal carotid artery or anterior cerebral arteries
13. Symmetric lesions in the temporal halves of both retinas, such as severe retinal edema
associated with diabetic retinopathy
14. Trauma
Cox TA, et al. Unilateral nasal hemianopia as a sign of intracranial optic nerve
compression. Am J Ophthalmol 1981; 92:230-232.
BITEMPORAL HEMIANOPIA
This condition involves defects in the temporal half of the visual field, usually
incomplete; it is due to pressure on the optic chiasm.
1. Chiasmal lesions
A. Congenital defect such as de Morsier syndrome (septooptic dysplasia);
autosomal dominant
*(1) Basal meningitis, including: chronic chiasmal syphilitic, tuberculous,
actinomycotic, and cysticercal arachnoiditis
(2) Chiasmal neuritis
C. Tumors of the chiasm
*(1) Primary tumors, including gliomas in childhood
*(2) Secondary tumors (rare), including meningiomas, retinoblastoma,
pinealoma, and ependymoma
D. Vascular lesions
(1) Arterial compression
(2) Arteriosclerosis
(3) Ectasia of the intracranial carotid arteries
*(4) Intracranial aneurysms, such as congenital, endocardial emboli,
traumatic, atheromatous, or syphilitic, especially intrasellar aneurysms
(5) Thrombosis of the carotid artery
2. Perisellar lesions
A. Parasellar tumors
(1) Injuries to the chiasmal pathway, such as from trauma
*(2) Meningioma of the sphenoid ridge
(3) Migraine
(4) Sudden onset without apparent cause
a. Arteriosclerotic or giant cell arteritic occlusion of nutrient
vessels of the chiasm in older patients
b. Disseminated sclerosis
(5) Tumors of the basal meninges
(6) Tumors of the sphenoid bone including osteochondroma, sarcoma,
anaplastic carcinoma
B. Presellar tumors
*(1) Meningioma of the olfactory groove
(2) Neuroblastoma of the olfactory groove
C. Suprasellar tumors
(1) Chordoma
*(2) Craniopharyngioma-manifestations may include diabetes insipidus,
infantilism, and calcification of hypophyseal-pituitary region
(3) Epidermoids
(4) Lymphoblastoma
(5) Pinealoma
*(6) Suprasellar meningioma
(7) Teratoma
(8) Tumors of the frontal lobe, including porencephaly (cystic cavity in
brain substance) and glioma
(9) Tumors of the third ventricle and internal hydrocephalus, such as
glioma and epidymoma
3. Pituitary lesions
A. Pituitary hyperplasia
B. Pituitary tumors
(1) Adenoma
a. Acidophilic adenoma-varies from gigantism to acromegaly
b. Basophilic adenoma-hyperadrenalism (Cushing disease), Nelson
syndrome
c. Chromophobe adenoma-varies from no endocrine symptoms to
panhypopituitarism; most common type of pituitary tumor,
Fröhlich syndrome
(2) Adenocarcinoma (rare)
(3) Metastatic tumors as from breast (rare)
Kerrison JB, Lee AG. Acute loss of vision during pregnancy due to a suprasellar mass.
Pomerantz HD, Lessell S. A hereditary chiasmal optic neuropathy. Arch Ophthalmol

1999; 117:128-131.
HOMONYMOUSQUADRANTANOPIA
In this condition, one quadrant is involved in upper or lower and right or left
visual fields; etiology may include tumor, vascular lesion, or infection.
1. Superior homonymous quadrantanopia

A. Inferior lip of the calcarine fissure-congruous
B. Temporal lobe-incongruous
2. Inferior homonymous quadrantanopia
A. Anton syndrome (denial-visual hallucination)
B. Superior radiation in parietal lobe-incongruous
C. Upper lip of the calcarine fissure in the occipital lobe-congruous
Bosley TM, et al. Neuro-imaging and positron emission tomography of congenital

homonymous hemianopsia. Am J Ophthalmol 1991; 111:413-418.
Harrington DO, Drake MV. The visual fields: textbook and atlas of clinical perimetry.
6th ed. St. Louis: CV Mosby, 1990.
CROSSED QUADRANTANOPIA
In this condition, the upper quadrant of one visual field is along with the lower
quadrant of opposite visual field.
* 1. Asymmetric homonymous hemianopia, such as vascular lesion of the upper lip of the
calcarine area on one side and the lower lip of the opposite calcarine cortex
2. Chiasm compression from lesion below compressing it against contiguous arterial
structure
3. Glaucoma
4. Inflammatory lesion, such as choroiditis juxtapapillaris
Harrington DO, Drake MV. The visual fields' text and atlas of clinical perimetry, 6th ed.
St. Louis: CV Mosby. 1990.
HOMONYMOUS HEMIANOPIA
This type of hemianopia affects the right or left halves of the visual fields; the
lesion is posterior to the optic chiasm,
1. Optic tract lesions-visual conduction system posterior to optic chiasm and anterior to
lateral geniculate body; lesion demonstrates incongruous field defect on side opposite to
defect, often with decreased vision, optic atrophy and afferent pupil.
A. Demyelinative disease-retrobulbar, multiple sclerosis, and Schilder disease
B. Migraine
C. Pituitary adenomas and craniopharyngiomas (most common); nasopharyngeal
carcinomas, chordomas, infundibulomas, and gliomas (less common)
D. Saccular aneurysms of internal carotid or posterior communicating artery
E. Trauma
2. Temporoparietal lesions-temporal lobe lesions are manifest initially in the upper visual
fields, whereas lesions of the parietal lobe are first manifest in the lower visual fields
A. Diffuse demyelinative diseases
(1) Krabbe type (Sturge-Weber-Krabbe syndrome)
(2) Metachromatic leukoencephalopathy
(3) Pelizaeus-Merzbacher type (aplasia axialis extracorticalis congenita)
(4) Progressive multifocal leukoencephalopathy
(5) Schilder type (encephalitis periaxialis diffusa)
(6) Spongy degeneration of the brain (Canavan disease)
B. Migraine
C. Tumor-gradual onset of symptoms-lesions include intrinsic astrocytoma and
glioblastoma, extrinsic meningioma, and lung metastasis
D. Vascular lesions-sudden onset
(1) Embolism-may be associated with rheumatic or arteriosclerotic heart
disease, bacterial endocarditis, myocardial infarction, or septic focus in
lungs
(2) Occlusion-middle cerebral occlusion affects primarily the arm and
face; anterior cerebral occlusion affects primarily the leg
(3) Subdural hematoma-spontaneous or following trauma
(4) Thrombosis-premonitory symptoms include unilateral blackouts in one
eye
E. Trauma (surgical)
3. Occipital lesions-congruous field defect and macular sparing most likely
A. Demyelinative disease
(1) Creutzfeldt-Jakob disease
(2) Krabbe type (Sturge-Weber-Krabbe syndrome)
(3) Metachromatic leukoencephalopathy
(4) Pelizaeus-Merzbacher type (aplasia axialis extracorticalis congenita)
(5) Progressive multifocal leukoencephalopathy
(6) Schilder type (encephalitis periaxialis diffusa)
(7) Spongy degeneration of the brain (Canavan disease)
B. Migraine
C. Poisons, such as carbon monoxide, digitalis, mescal, opium, lysergic acid
diethylamide
D. Trauma
(1) Direct-penetrating missiles and depressed bone fragments
(2) Indirect-general concussion syndrome
(3) Temporal lobectomy
E. Tumors-gradual onset of symptoms-lesions include intrinsic astrocytoma and
glioblastoma, extrinsic meningioma, and lung metastasis
F. Vascular lesion-sudden onset
(1) Arteriovenous anomalies
(2) Aneurysms (rare)
(3) Occlusion of posterior cerebral artery-thrombotic or embolic
(4) Subclavian steal syndrome, with reversal of blood flow through the
vertebral artery
Vargas ME, et al. Homonymous field defect as the first manifestation of Creutzfeldt-
Jakob disease. Am J Ophthalmol 1995; 119:497-504.
SPIRAL-FIELD DEFECTS
*1. Hysteria
2. Radiation therapy in or about the retina, optic nerve, and anterior visual pathways
Fitzgerald CR, et al. Radiation therapy in and about the retina, optic nerve, and anterior
visual pathway. Arch Ophthalmol 1981; 99:611-623.
DOUBLE HOMONYMOUS HEMIANOPIA

This condition involves peripheral constriction with small vertical steps above and
below fixation as a result of lesions of the occipital area and probable involvement of
striate cortex of both occipital lobes.
1. Bilateral central retinal artery occlusion

2. Bilateral central retinal vein occlusion
3. Bilateral vascular lesions involving a calcarine fissure
4. Increased intracranial pressure with shift of uncal portion of temporal lobe down over
edge of tentorium with compression of posterior cerebral arteries and infarction in
calcarine cortex.
5. Partial recovery from cortical blindness (see p. 632) from trauma, anoxia, carbon
monoxide poisoning, cerebral angiography, cardiac arrest, exsanguination, and other
similar conditions
6. Severe end-stage glaucoma
7. Severe trauma with massive brain damage as in depressed fracture of occiput
Harrington DO, Drake MV. The visual fields: text and atlas of clinical perimetry. 6th ed.
PART II
General Signs and Symptoms
19
Visual Disturbance
CONTENTS
Acquired myopia 619
Acquired hyperopia 622
Dysmegalopsia-optical illusions of size 623
Bilateral transient loss of vision 624
Amaurosis fugax 624
Sudden painless loss of visual acuity-one eye 626
Posttraumatic loss of vision 626
Decreased visual acuity 627
Bilateral blurring of vision 631
Cortical blindness (cerebral blindness) 632
Blindness in childhood 637
Binocular diplopia 638
Binocular triplopia (uniocular diplopia) 639
Diplopia following head trauma 639
Eccentric vision 640
Decreased dark adaptation 640
Astigmatism 641
Visual allesthesia 641
Visual acuity loss after glaucoma surgery 642
Sudden painful loss of vision 642
Sudden painless loss of vision-both eyes 642
Gradual painless loss of vision 642
ACQUIRED MYOPIA *
This condition comprises an error of refraction in which parallel rays of light
focus in front of the retina, usually producing blurred distant vision and clear near vision.
* 1. Conditions such as diabetes mellitus or nuclear sclerotic cataract in which there is

increased index of refraction of lens
*2. Refractive myopia-increased curvature of the refracting surfaces because of the
following:
A. Ciliary muscle spasm
(1) Functional-adolescence, hysteria
(2) Miotics such as carbachol, demecarium, echothiophate, isoflurophate,
neostigmine, and physostigmine
(3) Trauma-ocular contusion or anterior dislocation of the lens
(4) Mushroom (Amanita muscaria) poisoning
B. Lens hydration changes-diabetes mellitus, dysentery, or toxemia of pregnancy
C. Drug reaction-probably because of ciliary body edema, including the
following:
acetazolamide acetophenazine adrenal cortex injection
alcohol hyaluronidase polythiazide
aldosterone hydrochlorothiazide prednisolone
aspirin hydrocortisone prednisone
beclomethasone hydroflumethiazide prochlorperazine
bendroflumethiazide hydroxypropyl promazine
benzthiazide ibuprofen promethazine
betamethasone indapamide propiomazine
betaxolol isoflurophate quinethazone
butaperazine isosorbide dinitrate quinine
carbachol isotretinoin sodium salicylate
carphenazine levobunolol spironolactone
chlorothiazide medrysone sulfacetamide
chlorpromazine meprednisone sulfachlorpyridazine
chlortetracycline mesoridazine sulfacytine
chlorthalidone methacholine sulfadiazine
cimetidine (?) methacycline sulfadimethoxine
clofibrate methazolamide sulfamerazine
codeine methdilazine sulfameter
cortisone methotrimeprazine sulfamethazine
cyclothiazide methsuximide sulfamethizole
demecarium methyclothiazide sulfamethoxazole
demeclocycline methylcellulose sulfamethoxypyridazine
desoxycorticosterone methylprednisolone sulfanilamide
dexamethasone metolazone sulfaphenazole
dichlorphenamide minocycline sulfapyridine
diethazine morphine sulfasalazine
digitalis (?) neostigmine sulfathiazole
doxycycline opium sulfisoxazole
droperidol (?) oral contraceptives tetracycline
echothiophate oxygen thiethylperazine
ethopropazine oxytetracycline thiopropazate
ethosuximide paramethasone thioproperazine
ethoxzolamide penicillamine thioridazine
etretinate perazine timolol
fludrocortisone pericyazine triamcinolone
fluorometholone perphenazine trichlormethiazide
fluphenazine phenformin trifluoperazine
fluprednisolone phensuximide trifluperidol (?)
glibenclamide physostigmine triflupromazine
haloperidol (?) pilocarpine trimeprazine
hyaluronic acid piperacetazine
D. Elongated globe
E. Paralysis of accommodation for distance (sympathetic paralysis)-young patient
with unilateral Homer syndrome or migraine
F. Retinopathy of prematurity (retrolental fibroplasia)
G. Congenital glaucoma
H. Albinism
I. Gyrate atrophy (ornithine ketoacid aminotransferase deficiency)
J. Hypoparathyroidism
K. Malaria
L. Inherited
(1) Cochlear deafness with myopia and intellectual impairment-autosomal
recessive
(2) Epiphyseal dysplasia of femoral heads, myopia, deafness-autosomal
recessive
(3) Epiphyseal dysplasia, multiple, with myopia and conductive deafness-
autosomal dominant
(4) Microcornea and cataract-autosomal dominant
(5) Microphthalmos with myopia and corectopia-autosomal dominant
(6) Myopia-autosomal recessive or dominant or less often X-linked
(7) Night blindness, congenital stationary with myopia (nyctalopia-
myopia)-X-linked
(8) Night blindness with high-grade myopia-autosomal recessive
(9) Pinguecula blindness (total color blindness with myopia,
achromatopsia with myopia)-autosomal recessive
M. With scleral buckling surgery
3. Syndromes associated with myopia
A. Aberfeld syndrome (congenital blepharophimosis)
B. Achard syndrome (Marfan syndrome with mandibulofacial dysostosis)
C. Alport syndrome (hereditary familial congenital hemorrhagic nephritis)
D. Bloch-Sulzberger syndrome
E. Chromosome partial deletion (long-arm) syndrome
F. Cohen syndrome
G. Cri-du-chat syndrome
H. de Lange syndrome (congenital muscular hypertrophy cerebral syndrome)
I. Down syndrome (trisomy syndrome)
J. Ehlers-Danlos syndrome (fibrodysplasia elastica generalisata)
K. Fetal alcohol syndrome
L. Forsius-Eriksson syndrome (Aland disease)
M. Gansslen syndrome (familial hemolytic icterus)
N. Haney-Falls syndrome (congenital keratoconus posticus circumscriptus)
O. Homocystinuria
P. Hypomelanosis of Ito syndrome
Q. Kartagener syndrome (sinusitis, bronchiectasis, situs inversus syndrome)
R. Kniest syndrome
S. Laurence-Moon-Bardet-Biedl syndrome (retinitis pigmentosa-polydactyly-
T. Marchesani syndrome (brachymorphy with spherophakia)
U. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)
V. Marshall syndrome (atypical ectodermal dysplasia)
W. Matsoukas syndrome (oculocerebroarticuloskeletal syndrome)
X. Myasthenia gravis (Erb-Goldflam syndrome)
Y. Noonan syndrome (male Turner syndrome)
Z. Obesity-cerebral-ocular-skeletal anomalies syndrome
AA. Oculodental syndrome (Peter syndrome)
BB. Pierre Robin syndrome (micrognathia-glossoptosis syndrome)
CC. Pigmentary ocular dispersion syndrome
DD. Rubinstein-Taybi syndrome (broad-thumbs syndrome)
EE. SED congenita (spondyloepiphyseal dysplasia, congenital type)-autosomal
dominant
FF. Scheie syndrome
GG. Schwartz syndrome (glaucoma associated with retinal detachment)
HH. Siemens syndrome (hereditary ectodermal dysplasia syndrome)-autosomal
recessive
II. Smith-Magenis syndrome
JJ. Stickler syndrome (hereditary progressive arthroophthalmopathy)-autosomal
dominant
KK. Trisomy 20p syndrome
LL. Trisomy syndrome
MM. Tuomaala-Haapanen syndrome (unknown etiology, similar to
pseudohypoparathyroidism)
NN. Van Bogaert-Hozoy syndrome (similar to Rubinstein-Taybi syndrome)
OO. Wagner syndrome (hyaloideoretinal degeneration)
PP. Weill-Marchesani syndrome (brachymorphy with spherophakia)
QQ. Wrinkly-skin syndrome
RR. XXXXY syndrome (hypogenitalism, limited elbow pronation, low dermal
finger tip ridge count)
4. Transient myopia
A. Chemical agents and disease
*B. Diabetes
*C. After surgery
D. Trauma
Chow DR, et al. Refractive changes associated with scleral buckling and division in
retinopathy of prematurity. Arch Ophthalmol 1998; 116:1446-1450.
Finucane BM, Jaeger ER. Smith-Magenis syndrome. Ophthalmology 1997; 104:732-733.
Sorenson AL, et al. Ultrasonographic measurement of induced myopia associated with
capsular bag distention syndrome. Ophthalmology 2000; 107:902-908.
ACQUIRED HYPEROPIA
This condition comprises far-sightedness and error of refraction in which parallel
rays of light focus behind the retina, usually producing clear distant vision and blurred
near vision.
1. Aarskog syndrome (facial-digital-genital syndrome)

2. Adie syndrome (tonic pupil)
*3. Aphakia
4. Best syndrome (vitelliform dystrophy)
*5. Diabetes mellitus (poorly controlled to controlled)
6. Down syndrome (mongolism)
antihistamines sulfachlorpyridazine (?) sulfamethoxypyridazine
cannabis sulfadiazine (?) (?)
chloroquine sulfadimethoxine (?) sulfanilamide (?)
ergot sulfamerazine sulfaphenazole (?)
imipramine sulfameter (?) sulfapyridine (?)
meprobamate sulfamethazine sulfasalazine (?)
parasympatholytic drugs sulfamethizole sulfathiazole (?)
penicillamine sulfamethoxazole sulfisoxazole (?)
phenothiazine tolbutamide (?)
8. Flat cornea
9. Gorlin-Chaudhry-Moss syndrome (multiple basal cell nevi syndrome)
*10. Hyperopia-refractive or axial
11. Hypoglycemia
12. Kenny syndrome (nanophthalmos with hyperopia)
14. Lesions causing internal ophthalmoplegia with paralysis of accommodation
15. Macular edema
16. Orbital tumor with extraocular globe pressure and retinal striae
17. Postsurgical correction of myopia (retinal keratotomy, automated lamella
keratoplasty, photoreactive keratectomy)
*18. Presbyopia
19. Rubinstein-Taybi syndrome
20. Toxin of Clostridium botulinum
21. Trauma to the eye with posterior dislocation of the lens, macular edema, or ciliary
body contusion
John ME. High hyperopia after radial keratotomy. J Cataract Refract Surg 1993; 19:446-
448.
Newell FW. Ophthalmology: principles and concepts, 8th ed. St. Louis: CV Mosby.
1992.
DYSMEGALOPSIA-OPTICAL ILLUSIONS OF SIZE
1. Macropsia (objects appear larger)

*A. Miotics
*B. Spasm of accommodation (see p. 416-417)
C. Use of excessive plus lenses
2. Metamorphopsia (objects appear distorted)
A. Cerebral
(1) Drug intoxications
(2) Epilepsy
(3) Focal lesions such as thrombosis of right middle cerebral artery
*(4) Migraine
(5) Parietal lobe lesion, including tumor and vascular lesion
(6) Schizophrenia
B. Hysteria
C. Ocular
(1) Astigmatism
*(2) Macular lesions, including orbital tumor with macular striae and
macular edema, inflammation, heterotopia or hole
(3) Posterior vitreous separation and residual vitreoretinal macular traction
(4) Retinal detachment
D. Paget disease (osteitis deformans)
3. Micropsia (objects appear smaller)
A. Accommodative paralysis and subnormal accommodation
B. Atropinization
C. Botulism
D. Diphtheria
*E. Presbyopia
F. Use of excessive minus lenses
G. Use of scopolamine
4. Teleopsia (objects appear farther away than they actually are)
A. Bilateral parietal lesion
B. Parietal lesion in nondominant hemisphere
Saito Y, et al. The visual performance and metamorphopsia of patients with macular
holes. Arch Ophthalmol 2000; 118:41-44.
Walsh FB, Hoyt WF. Clinical neuro-ophthalmology, vol 1, 4th ed. Baltimore: Williams
& Wilkins, 1985.
BILATERAL TRANSIENT LOSS OF VISION (TRANSIENT DARKENING OF

VISION)
* 1. Circulatory disturbances when bending over or straining (postural hypotension)

2. Essential hypotension
A. Arteriosclerosis
B. Chronic hypotension
*C. Fatigue
D. Hormonal disorders
E. Hunger
F. Vitamin deficiency
3. Fainting with vasomotor collapse
4. Heart failure
5. Transurethral resection of the prostate
Creel DJ, et al. Transient blindness associated with transurethral resection of the prostate.
Arch Ophthalmol 1987; 105:1537-1539.
Levin LA, Moohta V. Postprandial transient visual loss. Ophthalmology 1997; 104:397-
401.
AMAUROSIS FUGAX (TRANSIENT MONOCULAR BLACKOUT OF VISION)
1. Amaurosis fugax syndrome

2. Arteriosclerosis, hypertension, and hypertensive crisis
3. Canalis opticus syndrome: functional-hysteria, neurasthenia
*4. Cerebrovascular insufficiency
A. Arterial aneurysms
B. Congenital or acquired arteriovenous malformations
C. Fibromuscular hyperplasia
D. Post-traumatic acute and chronic arterial occlusion
E. Takayasu syndrome (pulseless disease)
*F. Unilateral occlusive carotid disease
5. Compressive optic neuropathy
6. Corneal surface problems
7. Functional-hysteria, neurasthenia
8. Hematologic causes
A. Emboli
(1) Infective, such as subacute bacterial endocarditis
(2) Gas in dysbarism
B. Idiopathic thrombocytosis
C. Multiple myeloma (Kahler disease)
D. Polycythemia (Vaquez disease)
E. Severe anemia
F. Sickle cell disease (Herrick syndrome)
9. Hypotension of fundus
A. Cardiac arrhythmia
B. Glaucoma, narrow angle, pigment dispersion
C. Gaze-evoked amaurosis (transient monocular loss of vision occurring in a
particular direction of eccentric gaze)
*(1) Bone fragment adjacent to optic nerve following orbital fracture
*(2) Central retinal artery occlusion
(3) Optic nerve sheath meningiomas
(4) Orbital cavernous hemangiomas
(5) Orbital osteoma
B. Impending vascular occlusion, retinal vasospasm associated with systemic
vasospastic disease (migraine)
C. Increased intracranial pressure, such as from intracranial tumors that interfere
with vascular supply to the optic nerve
D. Increased venous pressure
(1) Impending central retinal vein occlusion (see p. 468-472)
(2) Intermittent elevation of intraocular pressure (glaucoma)
E. Negative G-force in pilots-circular maneuver with head toward the center of
the circle
F. Ocular ischemic syndrome
G. Orbital vascular insufficiency with giant cell arteritis
H. Papilledema-lasts for to seconds (see p. 593)
I. Positive G-force in pilots-circular maneuver with feet toward center of circle
J. Vasospasm including temporal arteritis, polyarteritis nodosa, eosinophilic
vasculitis, migraine and cluster headache
10. Large vitreous floater
11. Optic disc anomalies (congenital)
12. Ornithine transcarbamoylase deficiency
13. Pituitary tumor
15. Quinine poisoning
16. Raynaud disease (paroxysmal digital cyanosis)
17. Retrobulbar anesthesia
18. Schleral buckling procedure
19. Spontaneous bleeding from a normal-appearing iris or intraocular lens iris touch
20. Taveras syndrome (progressive intracranial arterial occlusion syndrome)
21. Uhthoff symptom-vision decreased with exercise or ocular hyperthermia can occur
with:
A. Friedreich ataxia
B. Insufficiency of posterior cerebral arteries
C. Intrasellar and parasellar tumor
D. Multiple sclerosis (disseminated sclerosis)
22. Uremic amaurosis-with eclampsia
23. Vasospasm
24. Wasp sting
Bernard GA, et al. Vasospastic amaurosis fugax. Arch Ophthalmol 1999; 117:1568.
Fineman MS, et al. Transient visual loss and decreased ocular blood flow velocities
following a schleral buckling procedure. Arch Ophthalmol 1999; 117:1647-1648.
Knapp ME, et al. Gaze-induced amaurosis from central artery compression.

Slavin M. Clinical challenges. Surv Opthalmol 1997; 41:6,481-487.
SUDDEN PAINLESS LOSS OF VISUAL ACUITY-ONE EYE
1. Acute keratoconus
2. Complication of retrobulbar block
3. Injury to the optic nerve
4. Meningeal carcinomatosis
5. Occlusion of central retinal artery
*6. Retinal detachment
7. Vitreous or retinal hemorrhage
Appen RE, et al. Meningeal carcinomatosis with blindness. Am J Ophthalmol 1978;

86:661-665.
Brookshire GL, et al. Life-threatening complication of retrobulbar block. Ophthalmology

1986; 93:1476-1478.
Kearne JR. Sudden blindness after ventriculography: bilateral retinal vascular occlusion
superimposed on papilledema. Am J Ophthalmol 1974; 78:275-278.
POSTTRAUMATIC LOSS OF VISION
*1. Acute (angle-closure) glaucoma precipitated by emotional trauma of recent accident

or from intumescent lens capsular trauma or other blunt trauma
2. Avulsion of optic nerve by lateral orbital wall trauma or contrecoup blow to head
3. Central retinal artery or vein occlusion (from markedly increased orbital pressure or
embolus)
4. Cortical blindness from hematoma, ischemia, or anoxia (patient may be unaware of
blindness)
5. Hyphema, vitreous hemorrhage
6. Hysteria
7. Indirect trauma to optic nerves or chiasm
8. Intracranial interruption of visual pathways (hemorrhage, foreign body)
*9. Lid swelling, blood or foreign material covering cornea, corneal damage
10. Malingering
*11. Retinal detachment
12. Traumatic cataract, luxation of the lens (see p. 401)
13. Traumatic retinal edema and hemorrhages of retina from direct or contrecoup blows
Deutsch TA, Feller DB. Paton and Goldberg's management of ocular injuries.
Philadelphia: WB Saunders, 1985.
DECREASED VISUAL ACUITY
1. Achromatopsia
2. Amblyopia ex anopsia-disuse
A. Anisometropia-difference in refractive error between the eyes
B. Monocular occlusion
C. Strabismus-esotropia, exotropia, or hypertropia
D. Unilateral atropinization
3. Anomalous elevation of optic disc with hyperplastic glial tissue and anomalous retinal
vessels
4. Apparently normal eye with central fixation with poorer visual acuity in one than other
eye-anisometropia
5. Apparently normal eye with normal fixation with disparity between near and distance
vision-amblyopia, hysteria, malingering, retrobulbar neuritis, presbyopia, and
micronystagmus
6. Apparently normal eye with normal fixation with poor distance and near vision
astigmatism, amblyopia, hyperopia in older persons
acebutolol albuterol aminosalicylic acid (?)
aceclidine alcohol amiodarone
acetaminophen aldosterone amithiozone
acetanilid alkavervir amitriptyline
acetazolamide allobarbital amobarbital
acetohexamide allopurinol amodiaquine
acetophenazine alprazolam amoxapine
acetyldigitoxin alseroxylon amphetamine
acid bismuth sodium aluminum nicotinate amphotericin B
tartrate (?) amantadine amyl nitrite
acyclovir ambenonium anisindione
adiphenine ambutonium anisotropine
adrenal cortex injection aminosalicylate (?) antazoline
antimony lithium butacaine chlorphenoxamine
thiomalate butalbital chlorphentermine
antimony potassium butallylonal chlorpromazine
tartrate butaperazine chlorpropamide
antimony sodium capreomycin chlorprothixene
tartrate captopril chlortetracycline
antimony sodium carbachol chlorthalidone
thioglycollate carbamazepine cimetidine
antipyrine carbinoxamine cisplatin
aprobarbital carbon dioxide clemastine
aspirin carbromal clidinium
atenolol carisoprodol clofazimine
atropine carmustine clofibrate
azatadine carphenazine clomiphene
bacille Calmette-Guérin cefaclor (?) clomipramine
(bCG) vaccine cefadroxil (?) clonazepam
bacitracin cefamandole (?) clonidine
baclofen cefazolin (?) clorazepate
barbital cefonicid (?) cobalt
belladonna cefoperazone (?) cocaine
bendroflumethiazide ceforanide (?) codeine
benoxinate cefotaxime (?) colchicine
benzathine penicillin G cefotetan (?) colloidal silver
benzphetamine cefoxitin (?) cortisone
benzthiazide cefsulodin (?) cryptenamine
benztropine ceftazidime (?) cyclizine
betamethasone ceftizoxime (?) cyclobarbital
betaxolol ceftriaxone(?) cyclopentobarbital
biperiden cefuroxime(?) cyclopentolate
bismuth oxychloride cephalexin(? ) cyclophosphamide
bismuth sodium tartrate cephaloglycin (?) cycloserine
(?) cephaloridine (?) cyclosporine
bismuth sodium cephalothin (?) cyclothiazide
thioglycollate (?) cephapirin (?) cycrimine
bismuth sodium cephradine (?) cyproheptadine
triglycollamate (?) chloral hydrate cytarabine
bismuth subcarbonate chlorambucil dacarbazine
(?) chloramphenicol danazol
bismuth subsalicylate (?) chlorcyclizine dantrolene
bromide chlordiazepoxide dapsone
bromisovalum chloroform deferoxamine
brompheniramine chloroprocaine demecarium
broxyquinoline chloroprocaine demeclocycline
bupivacaine chloroquine deserpidine
busulfan chlorothiazide desipramine
butabarbital chlorpheniramine deslanoside
desoxycorticosterone epinephrine hydrabamine
dexamethasone ergonovine penicillin V
dexbrompheniramine ergot hydralazine
dexchlorpheniramine ergotamine hydrochlorothiazide
dextroamphetamine erythrityl tetranitrate hydrocortisone
dextrothyroxine ethacrynic acid hydroflumethiazide
diatrizoate meglumine ethambutol hydromorphone
and sodium ethchlorvynol hydroxyamphetamine
diazepam ether hydroxychloroquine
diazoxide ethionamide ibuprofen
dibucaine ethopropazine imipramine
dichlorpbenamide ethosuximide indapamide
dicumarol ethoxzolamide indomethacin
dicyclomine etidocaine influenza virus vaccine
diethazine etretinate insulin
diethylpropion fenfluramine interferon
digitalis fenoprofen iodide and iodine
digitoxin flecainide solutions and
digoxin floxuridine compounds
diltiazem fludrocortisone iodochlorhydroxyquin
dimethindene fluorometholone iodoquinol
diphemanil fluorouracil iophendylate
diphenadione fluphenazine iothalamate meglumine
diphenhydramine fluprednisolone and sodium
diphenylpyraline flurazepam ophthalmic acid
diphtheria and tetanus flurbiprofen iron dextran
toxoids adsorbed furosemide isocarboxazid
diphtheria and tetanus gentamicin isoflurophate
toxoids and pertussis gitalin isoniazid
(adsorbed) glutethimide isopropamide
diphtheria toxoid glyburide isosorbide
(adsorbed) glycerin isosorbide dinitrate
dipivefrin glycopyrrolate isotretinoin
disopyramide griseofulvin kanamycin
disulfiram guanethidine ketamine
doxepin halazepam ketoprofen
doxycycline haloperidol labetalol
doxylamine hashish lanatoside C
dronabinol heparin levallorphan
droperidol heptabarbital levobunolol
dyclonine hexachlorophene levodopa
echothiophate hexamethonium levothyroxine
edrophonium hexethal lidocaine
emetine hexobarbital liothyronine
enalapril hexocyclium liotrix
ephedrine homatropine lithium carbonate
lomustine methyl alcohol orphenadrine
lorazepam methylatropine nitrate ouabain
lysergide methyldopa oxazepam
mannitol methylene blue oxprenolol
mannitol hexanitrate methylergonovine oxygen
maprotiline methylphenidate oxymorphone
marihuana methylprednisolone oxyphenbutazone
measles and rubella methyprylon oxyphencyclimine
virus vaccine (live) methysergide oxyphenonium
measles virus vaccine metoclopramide oxytetracycline
(live) metolazone paraldehyde
measles, mumps and metoprolol paramethasone
rubella virus vaccine metrizamide pemoline
(live) metronidazole pentaerythritol
mecamylamine mexiletine tetranitrate
mechlorethamine mianserin pentazocine
meclizine midazolam pentobarbital
medrysone minocycline pentolinium
mefenamic acid minoxidil perazine
melphalan mitomycin perhexiline
mepenzolate mitotane pericyazine
meperidine morphine perphenazine
mephenesin moxalactam (?) phenacaine
mephobarbital mumps virus vaccine phenacetin
mepivacaine (live) phencyclidine
meprednisone nadolol phendimetrazine
meprobamate nalidixic acid phenelzine
mescaline nalorphine phenindione
mesoridazine naloxone pheniramine
methacycline naltrexone phenmetrazine
methadone naphazoline phenobarbital
methamphetamine naproxen phensuximide
methantheline neostigmine phentermine
methaqualone niacin phenylbutazone
metharbital niacinamide phenylephrine
methazolamide nialamide phenylpropanolamine
methdilazine nicotinyl alcohol phenytoin
methitural nifedipine physostigmine
methixene nitrazepam pilocarpine
methocarbamol nitrofurantoin pimozide
methohexital nitroglycerin pindolol
methotrexate nitrous oxide pipenzolate
methotrimeprazine nortriptyline piperacetazine
methscopolamine nystatin piperazine
methsuximide opium piperidolate
methyclothiazide oral contraceptives piperocaine
piroxicam rubella and mumps virus tetrahydrozoline
poldine vaccine (live) thiabendazole
poliovirus vaccine rubella virus vaccine thiamylal
polymyxin B (live) thiethylperazine
polythiazide scopolamine thiopental
potassium penicillin G secobarbital thiopropazate
potassium penicillin V semustine thioproperazine
potassium phenethicillin silver nitrate thioridazine
practolol silver protein thiothixene
pralidoxime smallpox vaccine thyroglobulin
prazepam sodium thyroid
prazosin antimonylgluconate timolol
prednisolone sodium salicylate tobramycin
prednisone spironolactone tocainide
prilocaine stibocaptate tolazamide
primidone stibogluconate tolbutamide
probarbital stibophen tranylcypromine
procaine streptomycin trazodone
procaine penicillin G streptozocin triamcinolone
prochlorperazine sulfacetamide triazolam
procyclidine sulfachlorpyridazine trichlormethiazide
promazine sulfacytine trichloroethylene
promethazine sulfadiazine tridihexethyl
propanolol sulfadimethoxine triethylenemelamine
propantheline sulfamerazine trifluoperazine
proparacaine sulfameter trifluperidol
propiomazine sulfamethazine triflupromazine
propoxycaine sulfamethizole trihexyphenidyl
propoxyphene sulfamethoxazole trimeprazine
protoveratrines A and B sulfamethoxypyridazine trimethaphan
protriptyline sulfanilamide trimethidinium
psilocybin sulfaphenazole trimipramine
pyridostigmine sulfapyridine tripelennamine
pyrilamine sulfasalazine triprolidine
quinacrine sulfathiazole trolnitrate
quinethazone sulfisoxazole tropicamide
quinidine sulindac tryparsamide
quinine sulthiame uracil mustard
rabies immune globulin syrosmgopine urea
rabies vaccine talbutal urethan
radioactive iodides tamoxifen verapamil
ranitidine temazepam veratrum viride
rauwolfia serpentina tetracaine alkaloids
rescinnamine tetracycline vinbarbital
reserpine tetraethylammonium vinblastine
rifampin tetrahydrocannabinol warfarin
8. Hysteria
9. Irregular astigmatism-distortions in the anterior corneal surface (scarring, ectasia,
edema, ulcer, postinflammatory processes)
10. Macular pathology (including edema, hemorrhage, or scar tissue)
11. Malingering
*12. Myopia
13. Myotonic dystrophy-exertional vision loss
14. Nystagmus
15. Opacities of cornea, lens, or vitreous precluding good vision
16. Optic neuritis-retrobulbar and papillitis, including toxic causes such as those due to
tobacco, alcohol, and quinine (see p. 564)
17. Sphenoid sinus mucocele
18. Transient refractive errors
*A. Hyperopia
B. Myopia-diabetes
Cohen DB, Glasgow BJ. Bilateral optic nerve cryptococcosis in sudden blindness in
patients with acquired Immune deficiency syndrome. Ophthalmology 1993; 100:1689-
1694.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects. Woburn, MA.
Pau H. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical, 1988
BILATERAL BLURRING OF VISION
1. Drug-induced (see paresis of accommodation, p. 631-632)

2. Intracranial hypertension and advanced papilledema (see p. 593)
*3. Migraine-attacks last 15 to 20 minutes
4. Narcolepsy
5. Refractive error (myopia, hyperopia, presbyopia)
6. Retinal "blackout" experienced by pilots
7. Severe systemic hypertension
8. Systemic hypotension
9. Vertebrobasilar insufficiency
Norman ME, Dyer JA. Ophthalmic manifestations of narcolepsy. Am J Ophthalmol 1987;

103:81-86.
Wylie EJ, Ehrenfeld WK. Extracranial occlusive cerebrovascular disease: diagnosis and
management. Philadelphia: WB Saunders, 1970.
CORTICAL BLINDNESS (CEREBRAL BLINDNESS)

This condition involves a complete loss of all visual sensation, including all
appreciation of light and dark and a loss of reflex lid closure to bright illumination and to
threatening gestures; retention of pupil constriction to light and accommodation; normal
ophthalmoscopic examination; and normal motility. It may be associated with
hemiplegia, sensory disorders, aphasia, and disorientation.
1. Degenerative conditions
A. Alper progressive gray matter
B. Cerebral dysgenesis associated with dementia
C. Creutzfeldt-Jakob disease (corticostriatospinal degeneration)
D. Cytomegalic inclusion disease (rare)
E. Galactosemia
F. Hodgkin disease
G. Infantile neuroaxonal dystrophy
H. Krabbe syndrome
I. Phenylpyruvic oligophrenia
J. Pompe disease (generalized glycogenesis)
K. Porencephaly
L. Renal failure
M. Schilder disease (encephalitis periaxialis diffusa)
N. Scholz subacute cerebral sclerosis
O. Spongy degeneration of the brain
P. Subacute sclerosing panencephalitis
Q. Tay-Sachs disease (familial amaurotic idiocy)
R. Toxoplasmosis (rare)
alcohol hydrochlorothiazide (?) sulfachlorpyridazine
bendroflumethrazide (?) hydroflumethiazide (?) sulfacytine
benzthiazide (?) indaparnide (?) sulfadiazine
carbon dioxide iopamidol sulfadimethoxine
carbon monoxide iothalamate meglumine or sulfamerazine
chloroform (?) sodium iothalanuc sulfameter
chlorothiazide ketarnine (?) sulfamethazine
chlorthalidone lead poisoning sulfamethizole
cisplatin meglumine sulfamethoxazole
corticotropin methadone sulfamethoxypyridazine
cyclosporin methyclothiazide (?) sulfanilamide
cyclothiazide (?) methylergonovine (?) sulfaphenazole
diatrizoate meglumine or metolazone (?) sulfapyridine
sodium estradiol metrizamide sulfasalazine
ether (?) nifedipine sulfathiazole
etoposide nitroglycerin sulfisoxazole
etretinate nitrous oxide (7) tansy poisoning
FK506 polythiazide (7) thiopental (?)
glycine quinethazone trichlormethiazide (?)
hexamethonium chloride sulfacetamide vinblastine
vincristine
*3. Inflammatory lesions

A. Bacterial endocarditis
B. Encephalitis (including that due to measles and pertussis) and subacute
sclerosing panencephalitis
C. Influenza
D. Meningococcal meningitis
E. Mumps
F. Pneumococcal meningitis
G. Syphilitic meningitis
4. Space-taking lesions, such as tumors, gummas, abscesses, and cysts
*5. Trauma
A. Birth trauma, including heart dysfunction, postictal, and vertebral artery injury
B. Chiropractic manipulation of the neck and odontotic subluxation
C. Posthypoxic syndrome
D. Subdural hematoma with cerebral edema
E. Occipital region
F. Ventriculography and ventriculoatrial shunt operation
*6. Vascular lesions
A. Air embolism
B. Angioma of occipital region
C. Angiospastic lesions, including hypertension, nephritis, eclampsia, uremia, and
chronic lead poisoning (saturnism)
D. Anoxia from chronic respiratory insufficiency
E. Anoxia from high altitude
F. Basilar artery thrombosis
G. Bilateral posterior cerebral artery occlusion
H. Blood loss syndrome (acute cerebral hypotension)
I. Blood transfusion reaction
J. Cardiac arrest
K. Cerebral hemorrhage
L. Electroshock
M. Following bums and sunstroke
N. Following cardiac, cerebral or vertebral angiography
O. Hemorrhage in spastic paralysis
P. Herniation of hippocampal gyrus associated with subdural hematoma
Q. Hydrocephalus and microcephaly
R. Malaria
S. Obstruction of the local venous sinus, such as from septic thrombosis of
superior longitudinal sinus
T. Periarteritis nodosa
U. Subarachnoid hemorrhage
V. "Subclavian steal syndrome" with reversal of blood flow through the vertebral
artery
W. Thrombotic thrombocytopenic purpura
Rama BN. et al. Cortical blindness after cardiac catheterization: effect of rechallenge
with dye. Cathet Cardiovasc Diagn 1993:28:149-151.
Wong VC. Cortical blindness m children: a study of etiology and prognosis. Pediatr
Neurol 1991; 7:178-185.
Extracted Table Cortical Blindness
BLINDNESS IN CHILDHOOD
1. Cornea
A. Hereditary dystrophies
B. Inflammations such as varicella, rubeola, vaccinia, and gonorrhea, ophthalmia
neonatorum, and pemphigus
C. Trauma (abrasion or laceration)
2. Cortical blindness (see p. 632)
3. Globe
A. Anophthalmos (see p. 228)
B. Buphthalmos (see p. 222)
C. Congenital, primary infantile, or secondary glaucoma (see p. 305)
D. Hydrophthalmos
E. Microphthalmos (see p. 220)
4. Lens
A. Aphakia
B. Congenital cataracts (see syndromes associated with cataracts, p. 410-416)
5. Optic nerve
A. Aplasia
B. Asphyxia at birth
C. Associated with widespread disease such as mental deficiency, cerebral palsy,
or epilepsy
D. Atrophy (hereditary or secondary) (see p. 564)
E. Cavernous sinus thrombosis (Foix syndrome)
F. Cerebral hemorrhage (associated with major brain damage from accidental
trauma, abuse or birth trauma)
G. Crouzon syndrome (craniofacial dysostosis)
H. Hydrocephalus
I. Inflammatory damage-encephalomyelitis, encephalitis, tuberculous
J. Osteopetrosis (Albers-Schönberg syndrome)
K. Subdural hematoma
L. Trauma-fracture at the orbital canal
M. Tumors
6. Psychic blindness
A. Agnosia
B. Alexia
7. Retina
A. Achromatopsia
B. Albinism
C. Coats disease (retinal telangiectasia)
D. Early chorioretinal heredodegenerations, including Stargardt disease and
pigmentary retinopathy (see pseudoretinitis pigmentosa, p. 497-499)
E. Embryopathies, including rubella, toxoplasmosis, and syphilis
F. High myopia
G. Infantile macular degeneration
H. Pseudoretinitis pigmentosa (see p. 497-499)
I. Reese retinal dysplasia
J. Retinal detachment
K. Retinoblastoma
L. Retinoschisis
M. Retinopathy of prematurity
N. Tapetoretinal degeneration
8. Syndromes associated with amaurosis or blindness
A. Adie syndrome
B. Davidoff single ventricle
C. Laurence-Moon-Bardet-Biedl syndrome (retinitis-pigmentosa-polydactyly-
D. Malformative syndrome with cryptophthalmos
E. Marfan syndrome (arachnodactyly dystrophia mesodermalis congenita)
F. Metachromatic leukodystrophy (arylsulfatase A deficiency syndrome)
G. Niemann-Pick syndrome (essential lipoid histiocytosis)
H. Sandhoff disease
I. Schilder disease (encephalitis periaxialis diffusa)
9. Uveal tract
A. Chorioretinitis
B. Congenital coloboma
C. Iridocyclitis
10. Vitreous
A. Persistence of primary vitreous
B. Pseudoglioma
Brownstein S, et al. Sandhoff's disease (Gmgangliosidosis type 2). Arch Ophthalmol

1980; 98:1089.
Firth AY. Adie syndrome: evidence for refractive error and accommodative asymmetry
as the cause of amblyopia. Am J Ophthalmol 1999:128:118-119.
BINOCULAR DIPLOPIA (DOUBLE VISION USING BOTH EYES)
1. Intractable postoperative diplopia

A. Anomalous retinal correspondence with or without amblyopia (common),
which is called paradoxical diplopia
B. Cyclotropia due to oblique muscle operation
C. Following surgical treatment of retinal detachment because of symblepharon or
limitation of extraocular movement
D. "Horror fusionis" (rare)-congenital or developmental deficiency of fusion (i.e.,
absence of sensory correspondence between two eyes (not the same as abnormal
retinal correspondence, because visual directions are normal in these cases)
E. Large surgical overcorrection
2. Other
A. Aniseikonia, including association with macular disease
B. Divergence paresis
C. Heterophoria-due to lesions such as orbital tumor and cellulitis
D. Narcolepsy
E. Physiologic diplopia
F. Psychogenic causes
3. Paralysis of one or more extraocular muscles
A. Fourth-nerve palsy (rare) (see p. 158)
*B. Sixth-nerve palsy-has no localizing value (see p. 161)
C. Third-nerve palsy-with isolated muscle paralysis one must suspect a nuclear
lesion (hemorrhage, syphilis, multiple sclerosis) or myasthenia gravis (see p. 153)
Benegas NM, et al. Diplopia secondary to aniseikonia associated with macular disease.
Norman ME, Dyer JA. Ophthalmic manifestations of narcolepsy. Am J Ophthalmol 1987;

103:81-86.
Schanzer B, Bordaberry M. The child with divergence paresis. Surv Ophthalmol 1998;
42:571-574.
BINOCULAR TRIPLOPIA (UNIOCULAR DIPLOPIA)
1. Abnormal retinal correspondence with single image given two associations of direction
so that the abnormal retinal point is brought into consciousness at the same time as the
macula image
2. Central uniocular diplopia (rare)-systemic or neurologic causes include cerebral
aneurysm, abscess or gross degenerative lesions, encephalitis lethargica, postencephalitis,
multiple sclerosis, basal meningitis, cerebellar tumor, and vertebrobasilar insufficiency
*3. Malingering, hysteria, or psychogenic causes
4. Optical causes external to the eye
A. Double or single prism placed in center of pupil before one eye
*B. Improper correction of a high astigmatism
*C. Looking through the edge of a bifocal or margin of lens
5, Optical causes in the eye
A. Air bubbles or transparent foreign bodies in aqueous or vitreous
B. Complete or partial contraction of the eyelids in which the eyelids impinge on
the cornea (de Schweintz)
C Dislocation of the lens or misalignment of corneal and lenticular optical axis
D, Double pupil
E. High myopia, probably because of irregular astigmatism
F. Irregular astigmatism, such as pressure on the globe
G. Irregular spasm of the ciliary muscle
H Keratoconus (see p. 288)
I. Lens abnormalities, such as fluid clefts or incipient cataract
J. Looking through edge of intraocular lens
*K Map-dot fingerprint dystrophy
L Megalocornea (see p, 255)
M, Migration of filtering bleb into the cornea
N. Multifocal intraocular lens
O. Post iridectomy
*P. Refractive surgery
Q. Retinal detachment
R. Spherophakia (see p. 400-401)
Coffeen P, Guyton DL. Monocular diplopia accompanying ordinary refractive errors. Am

J Ophthalmol 1988; 105: 451-459.
Ellingson FT. Explanation of 3M diffractive intraocular lenses. J Cataract Refract Surg

1990; 16:697-702.
Girard LJ. Monocular diplopia accompanying ordinary refractive errors. Am J

Wyzinski P, O'Dell L. Subjective and objective findings after radial keratotomy.

Ophthalmology 1989; 96: 1608-1611.
DIPLOPIA FOLLOWING HEAD TRAUMA
1, Avulsion, contusion, or transection of extraocular muscles

2. Avulsion of the pulley of the superior oblique
3. Decompensation of a preexisting ocular phoria, becoming a tropia
4. Edema or detachment of the macula (monocular diplopia)
*5. Hematoma in the orbit or the ocular muscles
*6. Orbital fracture (particularly blowout fracture of the floor, causing restricted function
of inferior rectus and inferior oblique muscles)
7. Subluxation of the lens (monocular diplopia)
8. Third, fourth, or sixth cranial nerve palsies (orbital or intracranial) (see pp. 158, 161,
153)
9. "Whiplash" injury and the diplopias of obscure origin
Deutsch TA, Feller DB. Paton's and Goldberg's management of ocular injuries.
Philadelphia: WB Saunders, 1985.
ECCENTRIC VISION
In this condition, vision is best when the individual is not looking directly at
object of regard.
1. Central scotoma
2. Craniopharyngioma
3. Eccentric fixation with amblyopia
4. Ectopic macula, such as macula displaced by retinal scarring or fibrous strands, often a
result of retinopathy of prematurity
5. Glaucoma-late with only eccentric field remaining
6. Homonymous hemianopia with macular involvement (see p. 613)
*7. Macular scar, such as with age-related macular degeneration.
Beyer-Machule C, von Noorden GK: Atlas of ophthalmic surgery, vol 1: Lids, orbits,
extraocular muscles. New York: Thieme Medical. 1984.
Huber A. Eye signs and symptoms in brain tumors, 3rd ed. St. Louis: CV Mosby, 1976.
DECREASED DARK ADAPTATION (NYCTALOPIA; NIGHT BLINDNESS)
1. Choroideremia
2. Congenital night blindness
*3. May be due to drugs, including the following:
alcohol hashish methysergide
amodiaquine hydroxychloroquine oxygen
cantanidin indomethacin (?) pilocarpine
carbon dioxide isotretinoin psilocybin
chloroquine lithium carbonate silver nitrate
colloidal silver lysergic acid silver protein
deferoxamine diethylamide (LSD) tetrahydrocannabinol
dronabinol lysergide TI-iC
ergonovine marihuana vinblastine
ergotamine mescaline vincristine
etretinate methylergonovine
4. Progressive cone-rod dystrophy
5. Refsum syndrome (heredopathia atactica polyneuritiformis syndrome)
6. Retinitis pigmentosa (see p. 497)
Tasman W. Jaeger E. eds. Duane's clinical ophthalmology. Philadelphia: JB Lippincott,

1990.
ASTIGMATISM
In this condition, the refractive power of the eye varies along different meridians;
its steepest meridian is vertical in "with the rule" (corrected with plus cylinder at 90
degrees) and horizontal in “against the rule."
1. Adnexal masses
2. Anterior segment surgery for cornea, lens, or glaucoma
*3. Chalazion
*4, Contact lens wear
*5. Corneal scars
*6. Following refractive surgery
7. Keratoconus (see p. 288)
8. May be dominant inheritance with incomplete penetrance
9. Nuclear cataract with coloboma of lens, iris, and choroid
10. Oversized, rigid, anterior chamber, intraocular lens implant
11. Physiologic-about 0.5 diopters of "with the rule"
12. Retinal detachment procedures
13. Scleral infolding
14. Tilted intraocular lens
Abdel-Hakim AS. Corneal astigmatism induced by oversized rigid anterior chamber

implants. Am Intra Implant Soc J 1985; 11:474-482.
Bouzas AG. Anterior polar congenital cataract and corneal astigmatism. J Pediatr
Fraunfelder FT. Roy FH. Current ocular therapy, 5th ed Philadelphia: WB Saunders.
2000.
Hayashi H, et al Corneal shape changes after scleral buckling surgery. Opthalmology

1997; 104:831-837.
Inoue T, et al. Factors that influence the surgical effects of astigmatic keratotomy after
cataract surgery. Ophthalmology 2001; 108:1269-1274.
Kim T. et al. Induced corneal astigmatism after macular translocation surgery with scleral
infolding. Ophthalmology 2001; 108: 1203-1208
Plager DA, Snyder SK. Resolution of astigmatism after surgical resection of capillary
hemangiomas in infants. Ophthalmology 1997; 104:1102-1106.
VISUAL ALLESTHESIA
This condition involves displacement of image to opposite half of the visual field.
1. Parietooccipital lobe disease

A. Neoplasm
B. Vascular insufficiency
C. Trauma
D. Seizure activity
2. Occipital-lobe disease
A. Neoplasm
B. Vascular insufficiency
C. Trauma
D. Seizure activity
Bowen SF. Visual disorientation in allesthesia and palinopsia. JAMA 1978; 239:56.
Jacobs L. Visual allesthesia. Neurology 1980; 30: 1059.

VISUAL ACUITY LOSS AFTER GLAUCOMA SURGERY
1. Cystoid macular edema

*2. Hypotony maculopathy
3. Intraocular pressure spike
*4. Lens opacification
5. Postoperative capsule opacity
7. Suprachoroidal hemorrhage
8. Unknown
10. Wipeout (loss of central fixation)
Costa VP, et al. Loss of visual acuity after trabeculectomy. Ophthalmology 1993;
100:599-612.
Cristiansson J. Ocular hypotony after fistulizing glaucoma surgery. Acta Ophthalmol

1967; 45:837-845.
Watson RG, et al. The complications of trabeculectomy (a year follow-up). Eye 1990;
4:425-438.
SUDDEN PAINFUL LOSS OF VISION

1. Acute-angle closure glaucoma
2. Fracture of the lesser wing of the sphenoid bone
3. Keratoconus
4. Optic neuritis
6. Uveitis
Friedberg MA, Rapuano CJ. Office and emergency room diagnosis and treatment of eye
disease. Philadelphia: JB Lippincott, 1990.
SUDDEN PAINLESS LOSS OF VISION-BOTH EYES
1. Brain injury
2. Brainstem arteriovenous malformations
3. Meningeal carcinomatosis
4. Quinine poisoning
5. Wood alcohol poisoning (methyl)
Kerrison JB, Lee AG. Acute loss of vision during pregnancy due to a suprasellar mass.
GRADUAL PAINLESS LOSS OF VISION
1. Age-related macular degeneration

2. Bartonella henselae
3. Behcet disease
4. Cardiolipin antibody syndrome
5. Cataract
6. Chronic corneal disease
8. Eales disease
9. Glaucoma, open-angle
10. Herpetic viral infection
11. Idiopathic causes
12. Optic neuropathy/atrophy
13. Refractive error
14. Retinal disease, chronic
Goldstein SM. et al. Cancer-associated retinopathy. Arch Ophthalmol 1999; 117:1641-
l645.
20
Visual Complaint
CONTENTS
Photopsia 644
Hallucinations 645
"Spots" before eyes 648
Colored halos around lights 648
Light streaks 649
Photophobia 650
Asthenopia 652
Dazzling or glare discomfort 652
Chromatopsia 653
Heightened color perception 656
Nyctalopia (night blindness) 656
Hemeralopia (day blindness) 657
Oscillopsia 658
Color blindness 658
Palinopsia 661
Vertical reading 662
Visual agnosia 662
Ocular lateropulsion 662
Pain in and about eye 662
Headache 663
Pulfrich phenomenon 664
PHOTOPSIA (SCINTILLATIONS, SPARKS, OR FLASHES OF LIGHT BEFORE

THE EYES)
1. Associated with arteriovenous aneurysm

2. Auditory-visual synesthetic phenomena-optic nerve lesion; usually demyelinative
3. Brain concussion
4. Clomiphene citrate (Clomid)
5. Focal lesions of occipital region
6. Glaucoma
7. Idiopathic thrombocytosis
8. Impending retinal detachment
9. Lyme borreliosis
*10. Migraine and epilepsy
11. Moore lightning streak-traction of a partially liquefied vitreous on the retina
12. Oculodigital phenomenon (entopic phenomenon)
13. Paraneoplastic retinopathy
14. Phosphene of quick eye motion (Flick phosphene)
15. Retinal microembolization
16. Retinitis
17. Vertebral basilar insufficiency
Mikkila HA, et al. The expanding clinical spectrum of ocular Lyme borreliosis.
Pan H. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical, 1988.
Walsh FB, Hoyt WF. Clinical neuro-ophthalmology. 4th ed. Baltimore: Williams &
Wilkins, 1985.
HALLUCINA TIONS (FORMED IMAGES)
1. Blind persons (central or peripheral visual field loss)

2. Bilateral eye covering-such as may be required after an eye operation, especially in
older patients
3. Ocular lesions, such as retinal hemorrhage, glaucoma, optic atrophy of tertiary
syphilis, and choroidal neovascularization.
4. Psychoses
5. Central nervous system lesions
A. Alzheimer disease
B. Diffuse irritative lesion of parietotemporal area, including uncinate seizures of
the temporal lobe, stimulation of superior colliculus, and optic radiation
C. Encephalitis
D. Hippocampus lesions
E. Hypophyseal duct tumors
F. Measles
G. Medulloblastoma
H. Myxedema
I. Narcolepsy
J. Occipital lobe seizures-moving lights and colors, visual and complex
hallucinations with formed images
K. Papilledema (see p. 593)
L. Peduncular hallucinations with midbrain lesions from vascular, encephalitic,
and mass lesions
M. Pellagra
N. Pituitary and optic chiasmal lesion
O. Vertebrobasilar insufficiency/basilar artery migraine
6. Chronic mountain sickness (Monge syndrome)
8. Poisonings, such as mushroom, psilocin, cannabis, hashish, hemp, camphor, mescaline
from peyote, myristica (nutmeg), gasoline, mullet (Hawaiian fish), and ololiuqui
(morning-glory seeds)
acebutolol acid bismuth sodium albuterol
acetaminophen titrate alcohol
acetanilid acyclovir aldosterone
acetophenazine adrenal cortex injection allobarbital
alprazolam cefaclor cyclothiazide
amantadine cefadroxil cycrimine
amitriptyline cefamandole cyproheptadine
amobarbital cefazolin dantrolene
amodiaquine cefonicid dapsone
amoxapine cefoperazone demeclocycline
amphetamine ceforanide desipramine
amyl nitrite cefotaxime desoxycorticosterone
antazoline cefotetan dexamethasone
aprobarbital cefoxitin dexbrompheniramine
aspirin cefsulodin dexchlorpheniramine
atenolol ceftazidime dextroamphetamine
atropine ceftizoxime dextrothyroxine
azatadine ceftriaxone diazepam
baclofen cefuroxime diethazine
barbital cephalexin diethylpropion
belladonna cephaloglycin digitalis
bendroflumethiazide cephaloridine digoxin
benzathine penicillin G cephalothin diltiazem
benzphetamine cephapirin dimethindene
benztropine cephradine diphenhydramine
betamethasone chloral hydrate diphenylhydantoin
betaxolol chlorambucil diphenylpyraline
biperiden chlordiazepoxide disopyramide
bismuth oxychloride chloroquine disulfiram
bismuth sodium tartrate chlorpheniramine ditaven
bismuth sodium chlorphenoxamine divalproex sodium
thioglycollate chlorpromazine doxepin
bismuth sodium chlortetracycline doxycycline
triglycollamate chlorthalidone dronabinol
bismuth subcarbonate cholecalciferol droperidol
bismuth subsalicylate cimetidine enalapril
bromide clemastine ephedrine
brompheniramine clomipramine ergocalciferol
butabarbital clonazepam ethchlorvynol
butalbital clonidine ethionamide
butallylonal clorazepate ethopropazine
butaperazine cocaine ethosuximide
butethal codeine fenfluramine
calcitriol cortisone flecainide
capreomycin cyclizine fludrocortisone
captopril cyclobarbital fluphenazine
carbamazepine cyclopentobarbital fluprednisolone
carbinoxarnine cyclopentolate flurazepam
carbon dioxide cycloserine furosemide
carphenazine cyclosporine gentamicin
glutethimide mephobarbital oxprenolol
glycerin meprednisone oxyphenbutazone
griseofulvin mescaline oxytetracycline
halazepam mesoridazine paraldehyde
haloperidol methacycline paramethasone
hashish methamphetamine pargyline
heptabarbital methaqualone pemoline
hexethal metharbital penicillin
hexobarbital methdilazine pentazocine
homatropine methitural pentobarbital
hydrabamine methohexital pentylenetetrazol
penicillin V methotrimeprazine perazine
hydrochlorothiazide methscopolamine perhexiline (?)
hydrocortisone methsuximide pericyazine
hydroflumethiazide methyclothiazide perphenazine
hydroxy chloroquine methyldopa phenacetin
hydroxyurea methylpentynol phencyclidine
ibuprofen methylphenidate phendimetrazine
imipramine methylprednisolone phenelzine
indapamide methyprylon pheniramine
indomethacin methysergide phenobarbital
interferon metolazone phenoxymethyl
iodide and iodine metoprolol phenoxymethyl
solutions and metrizamide penicillin
compounds metronidazole phensuximide
isoniazid mexiletine phentermine
isosorbide mianserin phenylbutazone
ketamine midazolam phenylephrine
ketoprofen minocycline phenylpropanolamine
labetalol morphine (?) phenytoin
levallorphan moxalactam pimozide
levobunolol nadolol pindolol
levodopa nalidixic acid piperacetazine
levothyroxine nalorphine piperazine
lidocaine naloxone piroxicam
liothyronine naltrexone polythiazide
liotrix neostigmine potassium penicillin G
lithium carbonate nialamide potassium penicillin V
lorazepam nifedipine practolol
lysergide nitrazepam prazepam
mannitol nitrofurantoin (?) prazosm
maprotiline nitroglycerin (?) prednisolone
marihuana nortriptyline prednisone
meclizine opium (?) primidone
meperidine orphenadrine probarbital
mephentermine oxazepam procaine penicillin G
prochlorperazine sulfamethazine timolol
procyclidine sulfamethizole tobramycin
promazine sulfamethoxazole tocainide
promethazine sulfamethoxypyridazine trazodone
propiomazine sulfanilamide triamcinolone
propoxyphene sulfaphenazole triazolam
propranolol sulfapyridine trichlormethiazide
protriptyline sulfasalazine trichloroethylene
psilocybin sulfathiazole trifluoperazine
pyrilamine sulfisoxazole trifluperidol
quinacrine sulindac (?) triflupromazine
quinethazone talbutal trihexyphenidyl
quinidine temazepam trimeprazine
quinine tetanus immune globulin trimipramine
radioactive iodides tetanus toxoid tripelennamine
ranitidine tetracycline triprolidine
scopolamine tetrahydrocannabinol tropicamide
secobarbital thiabendazole urea
sodium salicylate thiamylal valproate sodium
sulfacetamide thiethylperazine valproic acid
sulfachlorpyridazine thiopental verapamil
sulfacytine thiopropazate vidarabine
sulfadiazine thioproperazine vinbarbital
sulfadimethoxine thioridazine vinblastine
sulfamerazine thyroglobulin vincristine
sulfameter thyroid vitamin D
10. Exercise induced with occipital lobe tumor

11. Patients in seclusion
Fisher CM. Visual hallucinations, atropine toxicity. Am J Ophthalmol 1991; 112:368.
Fraunfelder FT. Fraunfelder FW. Drug-induced ocular side effects. Woburn. MA:
Lerner AJ, et al. Concomitants of visual hallucinations in Alzheimer's disease. Neurology

1994; 44:523-527.
Loewenstein JI. Visual hallucinations in patients with choroidal neovascularization.

JAMA 1994; 272:243.
Scott IU, et al. Visual hallucinations in patients with retinal disease. Am J Ophthalmol
2001; 131:590-598.
"SPOTS" BEFORE EYES (DOTS OR FILAMENTS THAT MOVE WITH

MOVEMENT OF EYE)
*1. Vitreous opacities-muscae volitantes; associated with preretinal hemorrhage, myopia,
posterior vitreous detachment, or intraocular inflammations
2. Scotomatous defects
A. Retinal lesions
B. Myopia
3. Corneal foreign-body reflection/corneal opacity
4. Carbon tetrachloride poisoning
5. Migraine
Grant WM. Toxicology of the eye, 2nd ed. Springfield, IL: Charles C Thomas, 1974.
Vaughan D, et al. General ophthalmology, 14th ed. Norwalk, CT: Appleton & Lange,
1995.
COLORED HALOS AROUND LIGHTS (BLUE AND VIOLET ARE NEXT TO

THE STIMULATING LIGHT AND RED OUTERMOST)
1. Glaucoma
A. Acute-angle closure with stretching of corneal lamellae
B. Open-angle glaucoma-halo noted on awakening (intraocular pressure is highest
in the morning)
2. Mucus on the cornea
3. Corneal scar/corneal edema
4. Krukenberg spindle
5. Lens opacities
6. Vitreous opacities (see p. 429-430)
7. Any haze of ocular media
8. Drugs probably affecting corneal epithelium, including the following:
acetophenazine ethopropazine paramethadione
acetyldigitoxin ethylene diamine perazine
amiodarone fluorometholone pericyazine
amodiaquine fluphenazine perphenazine
amyl nitrite gitalin piperacetazine
butaperazine hydrocortisone prednisolone
carphenazine hydroxy chloroquine prochlorperazine
chlorine dioxide lanatoside C promazine
chloroquine medrysone promethazine
chlorpromazine mesoridazine propiomazine
cortisone methdilazine quinacrine
deslanoside methotrimeprazine sildenafil
dexamethasone methylprednisolone thiethylperazine
diethazine nitroglycerin thiopropazate
digitalis nitronaphthalene thioproperazine
digitoxin oral contraceptives thioridazine
digoxin ouabain trifluoperazine
triflupromazine trimethadione
trimeprazine water (sterile)
9. Physiologic halos-most common when lens acts as diffracting gradient

10. Too intense exposure to light, as in snow blindness
11. Asymmetric placement of the intraocular lens in relation to the pupillary aperture
Fraunfelder FT. Fraunfelder FW. Drug-induced ocular side effects. Woburn. MA:
Gabrieli CB, et al. Subjective visual halos after sildenafil (Viagra) administration.
Ophthalmology 2001; 108:877 -88l.
Mantyjarvi M. et al Ocular side effects of amiodarone. Surv Ophthalmol 1998; 42:360-

366.
LIGHT STREAKS
1. Cataracts
2. Contact lenses
3. Excessive tear meniscus
4. Intraocular lens scratches
5. Lashes
*6. Migraine
7. Posterior capsules-lens fibers and debris-filled corrugations
8. Rapid eye movements (especially in a dark environment)
9. Reflection off edge of intraocular lens
10. Reflection off manipulation holes of intraocular lens
* 11. Retinal break/tear or detachment
12. Spectacles
13. Windshields, windows
Holladay JT, et al. Diagnosis and treatment of mysterious light streaks seen by patients
following extracapsular cataract extraction. American Intraimplant Society Journal 1985;
11:21-23.
Holladay JT, et al. The optimal size of a posterior capsulotomy. American Intra implant
Society Journal 1985; 11: 18-20.
PHOTOPHOBIA (PAINFUL INTOLERANCE OF THE EYES TO LIGHT)
1. Aniridia
*2. Ocular, including conjunctivitis, keratitis, iritis, iridocyclitis, uveitis, and corneal,
lenticular, and vitreous opacities, Angelucci syndrome (critical allergic conjunctivitis),
acute hemorrhagic conjunctiva, and cone-dysfunction syndrome
3. Albinism
4. Total color blindness (achromatopsia)
5. Patients with corneal lesions having diseases characterized by photosensitization
(xeroderma pigmentosa, hydroa vacciniforme, and smallpox)
6. Systemic diseases, including botulism, cystinosis, erythropoietic porphyria,
hypoparathyroidism, rabies, psittacosis, and schistosomiasis
7. Toxic causes, including mercury poisoning
acetohexamide clonazepam fluorometholone
acetophenazine clorazepate fluorouracil
adiphenine dapiprazole fluoxetine hydrochloride
adrenal cortex injection hydrochloride fluphenazine
allobarbital deferoxamine fluprednisolone
alprazolam demeclocycline flurazepam
ambutonium desipramine fluvoxamine maleate
amiodarone dextran furosemide (?)
amitriptyline dextrothyroxine glyburide
amobarbital diacetylmorphine glycopyrrolate
amodiaquine diazepam (?) gold Au 198
anisotropine dicyclomine gold sodium thiomalate
antazoline diethazine gold sodium thiosulfate
atropine diethylcarbamazine guanethidine
auranofin digitalis halazepam
aurothioglucose digitoxin hashish
aurothioglycanide dimethyl sulfoxide hexethal
beclomethasone diphemanil hexobarbital
belladonna dipivalyl epinephrine hexocyclium
bendroflumethiazide (DPE) homatropine
benzthiazide dipivefrin hydralazine
betamethasone disopyramide hydroxyamphetamine
botulinum A toxin doxepin indomethacin
brimonidine tartrate doxycycline hydroxychloroquine
brinzolamide dronabinol ibuprofen
bromide edrophonium imipramine
brompheniramine enalapril indomethacin
butaperazine ethambutol iron dextran
capecitabine ethionamide iron sorbitex
captopril (?) ethopropazine isocarboxazid
carbon dioxide ethosuximide isoniazid
carphenazine ethotoin isopropamide
chlordiazepoxide ferrocholinate labetalol
chloroquine ferrous fumarate latanoprost
chlorpromazine ferrous gluconate levarterenol
chlorpropamide ferrous succinate levothyroxine
chlortetracycline ferrous sulfate liothyronine
cimetidine flecainide liotrix
clidinium floxuridine lithium carbonate
clomiphene fludrocortisone lorazepam
marihuana oxyphenbutazone scopolamine
mepenzolate oxyphencyclimine sildenafil citrate
mephenytoin oxyphenonium streptomycin
mesoridazine oxytetracycline temazepam
methacycline paramethadione tetanus immune globulin
methantheline perazine tetanus toxoid
methdilazine pericyazine tetracycline
methixene perphenazine thiethylperazine
methotrimeprazine phenelzine thiopropazate
methoxsalen phensuximide thioproperazine
methsuximide phenylbutazone thioridazine
methyl alcohol pipenzolate thyroglobulin thyroid
methylatropine nitrate piperacetazine tolazamide
methyldopa piperidolate tolbutamide
metoclopramide poldine tranylcypromine
metrizamide practolol trichloroethylene
metronidazole procarbazine tridihexethyl
midazolam prochlorperazine trifluoperazine
minocycline promazine triflupromazine
nalidixic acid promethazine trimeprazine
naltrexone propantheline trimethadione
nialamide propiomazine trioxsalen
norepinephrine protriptyline tripelennamine
norfloxacin quinacrine vancomycin
nortriptyline quinidine vinbarbital
oral contraceptives quinine vinblastine
oxazepam rabies immune globulin vincristine
oxprenolol rabies vaccine
9. Normal ocular findings with photophobia

A. Trigeminal neuralgia (Charlin syndrome)
B. Migraine
C. Neurasthenia
D. Meningitis
E. Subarachnoid hemorrhage
F. Acromegaly
G. Associated with hypophyseal tumor and craniopharyngioma
H. During and following retrobulbar neuritis
I. Acrodynia (Feer syndrome)
J. Following severe head injury
K. Hypoparathyroidism
L. Lesions of gasserian ganglion
M. Tumors of ophthalmic branch of the trigeminal nerve, such as neuroma,
middle fossa tumor, and posterior fossa tumor, such as meningioma or acoustic neuroma
N. Increased intracranial pressure, including subdural hematomas
11. Avitaminosis B (pellagra)
12. Chédiak-Higashi syndrome (anomalous leukocytic inclusions with constitutional
stigmata)
13. Danbolt-Closs syndrome (acrodermatitis enteropathica)
14. Elschnig syndrome (meibomian conjunctivitis)
15. Feer syndrome (acrodynia)
16. Falling syndrome (phenylketonuria)
18. Gradenigo syndrome (temporal syndrome)
19. Hanhart syndrome (pseudoherpetic keratitis)
20. Hartnup syndrome (niacin deficiency)
21. Hysteria
22. Infantile globoid cell leukodystrophy (Krabbe disease)
23. Keratodermia palmaris et plantaris
24. Photosensitivity and sunburn
25. Reiter syndrome (polyarthritis enterica)
ASTHENOPIA (UNCOMFORTABLE OCULAR SENSATION OR EYE ACHE)
1. Dazzling from bright light

2. Episcleritis or scleritis
3. Glaucoma
4. Iritis or iridocyclitis
5. Neurasthenia or hysteria
6. Passive congestion
7. Phoria or tropia
9. Sinus disease
10. Spasm from muscles held too long in a restricted position
11. Subclinical open-angle glaucoma
12. Uncorrected refractive errors, especially hyperopia or astigmatism
13. Unknown
14. Use of rniotics
15. Weak accommodation
Otto J. Asthenopia and wide-angle glaucoma. Glaucoma 1986; 8:75-77.
DAZZLING OR GLARE DISCOMFORT
1. Altered pupillary response

2. Asymmetric placement of the intraocular lens in relation to the pupillary aperture
3. Corneal scars or foreign bodies
4. Drugs, such as chloroquine, acetazolamide, or trimethadione (Tridione)
5. Emotional disorders
7. Idiopathic
8. Lenticular changes
Brems RN, et al. Posterior chamber intraocular lenses in a series of autopsy eyes. J
Cataract Refract Surg 1986: 12:367-375.
Vaughan D, et al. General ophthalmology, 12th ed. Norwalk, CT: Appleton & Lange.
1989.
CHROMATOPSIA (COLORED VISION, OBJECTS ARE ABNORMALLY

COLORED)
1. Blue color (cyanopsia)

acetyldigitoxin deslanoside methylene blue
alcohol digitalis nalidixic acid
amodiaquine digitoxin oral contraceptives
amphetamine digoxin ouabain
chloroquine gitalin quinacrine
combination products of hydroxyamphetamine sildenafil citrate
estrogens and hydroxychloroquine
progestogens lanatoside C
B. Pseudophakia
C. Optic atrophy of tertiary syphilis
2. Red color (erythropsia)
acetyldigitoxin gitalin sulfachlorpyridazine
atropine homatropine sulfacytine
belladonna lanatoside C sulfadiazine
deslanoside methylergonovine sulfadimethoxine
digitalis methysergide sulfamerazine
digitoxin naproxen sulfameter
digoxin ouabain sulfamethazine
ergonovine quinine sulfamethizole
ergotamine sulfacetamide sulfamethoxazole
sulfamethoxypyridazine sulfapyridine sulfisoxazole
sulfanilamide sulfasalazine sulthiame
sulfaphenazole sulfathiazole
B. Hysteria
C. Optic atrophy of tertiary syphilis
D. Vitreous or retinal hemorrhage (see p. 473-478)
E. Pseudophakia and aphakia
F. Snow blindness or blindness following electric shock
G. After working with green monochrome video display terminals
H. Welding arc maculopathy
3. Yellow color (xanthopsia)
acetaminophen deserpidine mild silver protein
acetanilid deslanoside nalidixic acid
acetophenazine diethazine nitrofurantoin (?)
acetyldigitoxin digitalis ouabain
allobarbital digitoxin pentobarbital
alseroxylon digoxin pentylenetetrazol
amobarbital dronabinol perazine
amodiaquine ethopropazine pericyazine
amyl nitrite fluorescein perphenazine
aprobarbital fluphenazine phenacetin
aspirin furosemide phenobarbital
barbital gitalin piperacetazine
bendroflumethiazide hashish polythiazide
benzthiazide heptabarbital primidone
butabarbital hexethal pro barbital
butalbital hexobarbital prochlorperazine
butallylonal hydrochlorothiazide promazine
butaperazine hydroflumethiazide promethazine
butethal hydroxychloroquine propiomazine
carbachol (?) indapamide quinethazone
carbon dioxide lanatoside C qumacrine
carphenazine manjuana rauwolfia serpentina
chloramphenicol mephobarbital rescinnamine
chloroquine mesoridazine reserpine
chlorothiazide methaqualone secobarbital
chlorpromazine metharbital silver nitrate
chlortetracycline methazolamide silver protein
chlorthalidone methdilazine sodium salicylate
cimetidine (?) methitural streptomycin
colloidal silver methohexital sulfacetamide
cyclobarbital methotrimeprazine sulfachlorpyridazine
cyclopentobarbital methyclothiazide sulfacytine
cyclothiazide metolazone sulfadiazine
sulfadimethoxine sulfathiazole thiopropazate
sulfamerazine sulfisoxazole thioproperazine
sulfameter syrosingopine thioridazine
sulfamethazine talbutal trichlonnethiazide
sulfamethizole tetrahydrocannabinol trifluoperazine
sulfamethoxazole tetrahydrocannabinol triflupromazine
sulfamethoxypyridazine (THC) trimeprazine
sulfanilamide thiabendazole vinbarbital
sulfaphenazole thiamylal vitamin A
sulfapyridine thiethylperazine
sulfasalazine thiopental
B. Lenticular change
C. Aphakia
D. Poisons, including aconite, dichlorodiphenyl trichloroethane, carbon disulfide,
chromic acid, methyl salicylate, aspidium (Felix mas), santonin, picric acid
E. Jaundice
F. Hysteria
4. Green color (chloropsia)
acetyldigitoxin digoxin nalidixic acid
allobarbital epinephrine naproxen
amobarbital gitalin ouabain
amodiaquine griseofulvin pentobarbital
aprobarbital heptabarbital phenobarbital
barbital hexethal primidone
butabarbital hexobarbital probarbital
butalbital hydroxychloroquine quinacrine
butallylonal iodide and iodine quinine
butethal solutions and radioactive iodides
chloroquine compounds secobarbital
cyclobarbital lanatoside C talbutal
cyclopentobarbital mephobarbital thiamylal
deslanoside metharbital thiopental
digitalis methitural vinbarbital
digitoxin methohexital
B. Poisons such as santonin

5. Violet color (ianthinopsia)
dronabinol marihuana quinacrine
hashish nalidixic acid tetrahydrocannabinol
B. Pseudophakia and aphakia

C. Intracameral air
6. Brown color
acetophenazine methdilazine THC
butaperazine perazine thiethylperazine
carphenazine pericyazine thiopropazate
chlorpromazine perphenazine thioproperazine
diethazine piperacetazine thioridazine
ethopmpazine prochlorperazine trifluoperazine
fluphenazine promazine triflupromazine
inethotrimeprazine promethazine trimeprazine
mesoridazine propiomazine
B. Lenticular change
7. White color
capreomycin paramethadione phenytoin
diphenylhydantoin trimethadione
B. Pseudophakia and aphakia
Fraunfelder FT. Fraunfelder FW Drug-induced ocular side effects. Woburn, MA:

Uniat L, et al. Welding arc maculopathy. Am J Ophthalmol 1986:102:394-395.
Walsh FB, Hoyt WF. Clinical neuro-ophthalmology, vol 1, 4th ed. Baltimore: Williams
& Wilkins, 1985.
HEIGHTENED COLOR PERCEPTION
1. Heightened color perception is due to drugs, including the following:

dronabinol lysergide psilocybin
ethionamide marihuana tetrahydrocannabinol
hashish mescaline THC
LSD oxygen
NYCTALOPIA (NIGHT BLINDNESS)
1. Anemia
2. Carbon monoxide poisoning
3. Congenital high myopia
4. Diffuse opacities of media including corneal edema, keratitis and nuclear cataract
6. Glaucoma-especially open-angle and angle-closure glaucoma
7. Paraneoplastic retinopathy including melanoma-associated retinopathy and cancer-
associated retinopathy
8. Psychologic causes-malingering or psychoses
9. Optic atrophy
11. Tapetoretinal degenerations
A. Choroideremia
B. Congenital night blindness
(1) Dominant form
(2) Recessive form
(3) Recessive, sex-linked
C. Detachment of retina, including malignant melanoma
acetophenazine mesoridazine propiomazine
amodiaquine methdilazine quinidine
butaperazine methotrimeprazine quinine
carphenazine paramethadione thiethylperazine
chloroquine perazine thiopropazate
chlorpromazine pericyazine thioproperazine
diethazine perphenazine thioridazine
ethopropazine piperacetazine trifluoperazine
fluphenazine prochlorperazine triflupromazine
hydroxychloroquine promazine trimeprazine
indomethacin promethazine trimethadione
E. Drusen (familial)-minimal
F. Fleck retina-nonprogressive, congenital, rare
G. Fundus flavimaculatus-minimal
H. General choroidal sclerosis
I. Gyrate atrophy
J. Retinitis pigmentosa
K. Retinitis punctata albescens
L. Miner nystagmus
M. Oguchi disease-may be abnormal
12. Visual field defects
A. Dietary deficiencies, including malnutrition, alcoholism and cystic fibrosis
B. Digestive tract disturbance
(1) Colitis and enteritis
(2) Crohn disease
(3) Jejunoileal bypass surgery
(4) In pancreas-such as chronic pancreatitis
(5) In stomach-achlorhydria, chronic gastritis or diarrhea, peptic ulcer
(6) Abetalipoproteinemia
C. Liver disease, such as chronic cirrhosis
D. Malaria
E. Pregnancy
F. Pulmonary tuberculosis
G. Skin disorders, such as pityriasis rubra pilaris
H. Thyroid gland disorders, such as hyperthyroidism
14. Vitreous opacities, including hemorrhage
15. Vitreotapetoretinal degeneration-sex-linked recessive and autosomal recessive
Berson EL, Lessell S. Paraneoplastic night blindness with malignant melanoma. Am J

Ophthalmol 1988; 106: 307-311.
Dryja TP. Molecular genetics of Oguchi disease, fundus albipunctatus, and other forms of
stationary night blindness: LVII Edward Jasckson Memorial Lecture. Am J Ophthalmol
2000:130:547-563.
Gans M, Taylor C. Reversal of progressive nyctalopia in a patient with Crohn' disease.

Can J Ophthalmol 1990: 25:156-158.
HEMERALOPIA
This condition involves day blindness, that is, an inability to see as distinctly in a
bright light as in a dim one.
1. Adie pupil
2. Albinism
3. Aniridia
4. Central opacities of the lens-nuclear or perinuclear cataracts
5. Central scotoma
6. Congenital-autosomal recessive trait usually associated with amblyopia and color
deficiency
7. Hereditary retinoschisis
8. Intraocular iron
9. Partial occlusion of the central retinal artery (see p. 457-461)
11. Total color blindness
Gehrs K, Tiedeman J. Hemeralopia in an older adult. Surv Ophthalmol 1992:37:185-189.
Pau H. Differential diagnosis of eye diseases, 2nd ed. New York: Thieme Medical. 1988.
OSCILLOPSIA
This condition involves illusionary movement of the environment; it may be
unilateral or bilateral and usually occurs because of acquired nystagmus.
alcohol cyclopentobarbital phenobarbital
allobarbital diphenylhydantoin primidone
amobarbital gentamicin probarbital
aprobarbital heptabarbital secobarbital
barbital hexethal talbutal
butabarbital hexobarbital thiamylal
butalbital mephobarbital thiopental
butallylonal metharbital valproate sodium
butethal methitural valproic acid
carbamazepine methohexital vinbarbital
cyclobarbital pentobarbital
2. Fixation and voluntary nystagmus

3. Defective vestibuloocular reflex/vestibular pathway lesion occurs during movement of
the head or body
A. Sectioning of vestibular (VIII) nerve for vertigo
B. Streptomycin toxicity
C. Spontaneous loss
4. Head trauma/seizures
5. Intermittent exotropia
6. Involvement of medial longitudinal fasciculus affecting ipsilateral medial rectus in
internuclear ophthalmoplegia-monocular oscillopsia
7. Myokymia of the eyelid
8. Opsoclonus and ocular flutter
9. Vertebral artery dissection
Chrousos GA, et al. Two cases of downbeat nystagmus and oscillopsia associated with
carbamazepine. Am J Ophthalmol 1987; 103:221-224.
Hertle RW, et al. Onset of oscillopsia after visual maturation in patients with congenital
nystagmus. Ophthalmology 2001; 108:2301-2308.
Hicks PA, et al. Ophthalmic manifestation of vertebral artery dissection: patients seen at
Mayo Clinic from 1976-1992. Ophthalmology 1994; 10 1:1786-1792.
COLOR BLINDNESS
1. Inherited-stable defect, affecting both eyes

A. Bassen-Kornzweig syndrome (abetalipoproteinemia)
B. Congenital dyslexia syndrome (developmental dyslexia syndrome)
C. Down syndrome (mongolism)
D. Duane retraction syndrome (Stilling syndrome)
E. Duchenne muscular dystrophy
F. Glucose-6-phosphate dehydrogenase deficiency (glycogen storage disease type
I)
G. Guillain-Barré syndrome (acute infectious neuritis)
H. Hemophilia
I. "Intrinsic" defect
(1) Dichromat-two colors mixed to see white
a. Deuteranope-green deficiency
b. Protanope-red deficiency
c. Tritanope-blue deficiency
(2) Monochromat-one color mixed to see white
a. Cone deficient
b. Rod deficient
(3) Trichromat-three colors mixed to see white
a. Deuteranomaly-green anomaly
b. Protanomaly-red anomaly
c. Tritanomaly-blue anomaly
J. Kallman syndrome (hypogonadotrophic hypogonadism-anosmia syndrome)
K. Klinefelter syndrome (XXY) (gynecomastia-aspermatogenesis syndrome)
L. Turner syndrome (XO) (gonadal dysgenesis)
2. Acquired-defect can increase or decrease; may affect only one eye; impairment of
other visual function; often characterized by chromatopsia; hue discrimination primarily
affected; yellow-blue defects more common in retinal disease; red-green defects in optic
nerve disease
A. Advanced hypertensive retinopathy
B. Albinism
C. Amblyopia
D. Blue-yellow defect with retinal disorders from drugs, including the following:
acetophenazine clofazimine methotrimeprazine
amiodarone (?) clonidine (?) minoxidil (?)
amodiaquine cobalt (?) mitotane
azathioprine cycrimine (?) naproxen (?)
benztropine (?) deferoxamine penicillamine
biperiden (?) diethazine perazine
butaperazine diethylcarbamazine pericyazine
carbamazepine ethambutol perphenazine
carphenazine ethopropazine piperacetazine
cephaloridine (?) fluphenazine prazosin (?)
chloramphenicol hydroxychloroquine prochlorperazine
chloroquine indomethacin (?) procyclidine (?)
chlorphenoxamine (?) ketoprofen (?) promazine
chlorpromazine mesoridazine promethazine
chlorprothixene methdilazine propiomazine
cisplatin methotrexate quinacrine (?)
quinine thiopropazate trifluoperazine
sulindac (?) thioproperazine triflupromazine
tamoxifen thioridazine trihexyphenidyl (?)
thiethylperazine thiothixene trimeprazine
E. Chorioretinitis
F. Color anomia-inability to name colors; may be associated with homonymous
hemianopia resulting from infarct of posterior parietal and corpus callosum
G. Diabetic retinitis
H. Dominantly inherited juvenile optic atrophy
I. Drugs and chemical substances causing optic neuropathy with red-green defect,
including the following:
acetophenazine chloroprocaine (?) hexethal
alcohol chloroquine hexobarbital
allobarbital chlorpromazine hydrocortisone
alseroxylon (?) cholecalciferol hydroxychloroquine
aminosalicylate (?) clindamycin iodide and iodine
aminosalicylic acid (?) cobalt (?) solutions and
amobarbital cortisone compounds
amodiaquine cyclobarbital iodochlorhydroxyquin
antimony lithium cyclopentobarbital iodoquinol
thiomalate cycloserine (?) iron dextran (?)
antimony potassium dapsone iron sorbitex (?)
tartrate deferoxamine isoniazid
antimony sodium deserpidine (?) levothyroxine (?)
tartrate dexamethasone lidocaine (?)
antimony sodium dextrothyroxine (?) liothyronine(?)
thioglycollate diethazine liotrix (?)
antipyrine ergocalciferol medrysone
aprobarbital ergonovine (?) mephobarbital
aspirin ergot (?) mepivacaine (?)
barbital ergotamine (?) mesoridazine
betamethasone ethambutol metharbital
bromide (?) ethopropazine methdilazine
bromisovalum etidocaine (?) methitural
broxyquinoline ferrocholinate (?) methohexital
bupivacaine (?) ferrous fumarate (?) methotrexate (?)
butabarbital ferrous gluconate (?) methotrimeprazine
butalbital ferrous succinate (?) methyl alcohol
butallylonal ferrous sulfate (?) methylene blue
butaperazine fluorometholone methylergonovine
butethal fluphenazine methysergide (?)
calcitriol gentamicin nitroglycerin (?)
carbromal heptabarbital oxyphenbutazone
carphenazine hexachlorophene pentobarbital
chloramphenicol hexamethonium perazine
pericyazine sodium sulfisoxazole (?)
perphenazine antimonylgluconate suramin
phenobarbital sodium salicylate syrosingopine (?)
phenylbutazone stibocaptate talbutal
piperacetazine stibogluconate thiamylal
polysaccharide-iron stibophen thiethylperazine
complex (?) streptomycin thiopental
prednisolone sulfacetamide (?) thiopropazate
prilocaine (?) sulfachlorpyridazine (?) thioproperazine
primidone sulfacytine (?) thioridazine
probarbital sulfadiazine (?) thyroglobulin (?)
procaine (?) sulfadimethoxine (?) thyroid (?)
prochlorperazine sulfamerazine (?) tobramycin
promazine sulfameter (?) trichloroethylene
promethazine sulfamethazine trifluoperazine
propiomazine sulfamethizole (?) triflupromazine
propoxycaine (?) sulfamethoxazole (?) trimeprazine
propoxyphene sulfamethoxypyridazine tryparsamide
quinine (?) vinbarbital
radioactive iodides sulfanilamide (?) vinblastine
rauwolfia serpentina (?) sulfaphenazole (?) vincristine
rescinnamine (?) sulfapyridine (?) vitamin A
reserpine (?) sulfasalazine (?) vitamin D
secobarbital sulfathiazole (?)
J. Friedreich ataxia
K. Glaucoma, including narrow and open angle
L. Hepatic cirrhosis
M. Hysteria
N. Macular lesions, including juvenile degeneration, senile degeneration
dystrophy, and edema
O. Night blindness
P. Occlusion of retinal vessels
Q. Oguchi disease
R. Open-angle glaucoma
S. Ophthalmologist who use argon blue-green lasers or operating microscopes
T. Optic atrophy
U. Optic pathways, including brain tumor
V. Papillitis
W. Peripheral chorioretinal degeneration
X. Retinal detachment
Y. Retinitis pigmentosa
Z. Retrobulbar optic neuritis
AA. Snow blindness
Nousiainen I, et al. Color vision in epilepsy patients treated with vigabatrin or

carbamazepine monotherapy. Ophthalmology 2000; 107:884-888.
Sample PA, et al. Isolating the color vision loss in primary open angle glaucoma. Am
Ophthalmol 1988; 106:686-691.
PALINOPSIA
This condition involves persistence or recurrence of visual images after exciting
stimulus object has been removed; the patient has a hemianopic field defect. Polyopia
(visual trailing effect with movement) may be present.
1. Acute migraine
2. Demyelinative optic neuritis
3. Encephalitis
4. Epilepsy
5. Intoxications, such as mescal delirium, LSD, trazodone-induced and clomiphene
citrate.
6. Kartagener syndrome
7. Laser treatment of diabetic macular edema
8. Leber hereditary optic neuropathy
9. Temporal-parietal-occipital lesion
A. Degenerative
B. Neoplastic
C. Traumatic
D. Vascular
10. Schizophrenia
11. Drug, such as nefazodone (akinetopsia-persistence of moving objects)
Horton JC, Trobe JD. Akinetopsia from nefazodone toxicity. Am J Ophthalmol 1999;
128, 4:530-531.
Kawasaki A, Purvin V. Persistent palinopsia following ingestion of LSD. Arch

Ophthalmol 1996; 114:47-50.
Marneros A, Korner J. Chronic palinopsia in schizophrenia. Psychopathology 1993;

26:236-239.
Pomeranz HD, Lessell S. Palinopsia and polyopia in the absence of drugs or cerebral
disease. Neurology 2000; 54: 855-859.
Purvin VA. Visual disturbance secondary to clomiphene citrate. Arch Ophthalmol 1995;
113:482-484.
VERTICAL READING (PATIENT READS FROM ABOVE DOWNWARD)
1. Astigmatism-high error of refraction

2. Homonymous hemianopia (see p. 613)
3. Voluntary as oriental script written vertically
VISUAL AGNOSIA
This condition involves a failure to recognize objects by sight for animate and
inanimate objects, but ot does not interfere with recognition of language symbols.
1. Drugs
2. Kluver-Bucy syndrome (temporal lobectomy behavior syndrome)
3. Lesion of Brodmann area 18
Scheie HG. Textbook of ophthalmology, 10th ed. Philadelphia: WB Saunders, 1986.
OCULAR LATEROPULSION
The eyes feel as though they are being drawn toward one side, but this problem
can be overcome with conscious effect; full range of extraocular muscle movements is
maintained.
1. Lateral medullary disease, including infarction of lateral medullary plate, acoustic

neurinoma, posterior fossa meningioma, or multiple sclerosis
2. Peripheral vestibular disease
Meyer KT, et al. Ocular lateropulsion. Arch Ophthalmol 1980; 98:1614-1616.
PAIN IN AND ABOUT EYE
1. Ocular
A. Angle-closure glaucoma
B. Chronic ocular hypoxia, carotid occlusive disease
C. Dry-eye and tear-deficiency syndrome
D. Local lid, conjunctival, and anterior segment disease
E. Ocular inflammation including lyme borreliosis
2. Ophthalmic division
A. Herpes zoster
B. Migraine, cluster headache
C. Painful ophthalmoplegia syndrome
D. Raeder paratrigeminal neuralgia
E. Referred (dural) pain, including occipital infarction
F. Sinusitis
G. Tic douloureux (infrequent in VI)
3. Mandibular division
A. Dental disease
B. Tic douloureux
4. Maxillary division
A. Dental disease
B. Nasopharyngeal carcinoma
C. Sinusitis
D. Temporomandibular syndrome
E. Tic douloureux
5. Miscellaneous
A. Atypical facial neuralgias
B. Cranial arteritis
C. Pain with medullary lesions
D. Trigeminal tumors
Hill LM, Hasting G. Carotidynia: a pain syndrome. J Family Practice 1994; 39:71-75.
HEADACHE
1. Vascular headache of migraine type

A. Cephalalgia migraine (migraine equivalent)-migraine aura without headache
B. Classic migraine-migraine with aura
C. Common migraine-migraine without aura
D. Complicated migraine-hemiplegic migraine and ophthalmoplegia migraine
E. Cluster headache
F. Lower-half headache
2. Muscle-contraction headache
3. Combined (skeletal vascular)
4. Headache of nasal vasomotor reaction
5. Headache of delusional, conversion, or hypochondriacal states
6. Nonmigraine vascular headaches
A. Primary or metastatic tumors of meninges, vessels, or brain
B. Hematomas (epidural, subdural, or parenchymal)
C. Abscesses (epidural, subdural, or parenchymal)
D. Post lumbar puncture headache (leakage, headache)
E. Pseudomotor cerebri and various causes of brain swelling
7. Headache due to overt cranial inflammation
A. Intracranial disorders
(1) Mass
(2) Meningitis
(3) Subarachnoid hemorrhage
B. Extracranial disorders (temporal arteritis)
8. Headache because of diseases of ocular structures
9. Headache because of diseases of aural structures
10. Headache because of diseases of the nasal and sinus structures
11. Headache because of diseases of dental structures
12. Headache because of diseases of other cranial and neck structures
13. Cranial neuritides
14. Cranial neuralgia
A. Glossopharyngeal neuralgia
B. Trigeminal neuralgia
15. Analgesic/ergotamine rebound headache
Friedman AP, et al. Classification of headache. Neurology 1962; 12:173.
Mikkila HA, et al. The expanding clinical spectrum of ocular lyme borreliosis.
PULFRICH PHENOMENON
This condition involves a three-dimensional illusion in which a moving object
that is viewed binocularly with a light attenuating filter in front of one eye appears to
transcribe an anomalous pathway.
1. Age-related macular degeneration

2. Anisocoria
A. Induced
B. Traumatic
3. Anisometropic amblyopia
4. Cataract
5. Central serous retinopathy
6. Corneal Opacity
7. Hemianopia
8. Multiple Sclerosis
9. Optic Neuritis
10. Postretinal detachment repair
Larkin EB, Dutton GN, Heron G. Impaired perception of moving objects after minor
injuries to the eye midface: the pulfrich phenomenon. Br J Oral Maxillofac Surg 1994;
32:360-362.
Rubin ML. Perspectives in refraction. Surv Opthalmology 1997; 41:6,493-499.
Scotcher SM, Canning CR, Weal MJ, et al. Pulfrich's phenomenon in patients with
unilateral cataract: a previously unrecognized cause of visual disability. Invest Opthalmol
Vis Sci 1995; 36[Suppl]:s794 (abst).
21
Head Position
CONTENTS
Head turn (face turn) 665
Head tilt 666
Chin elevation 667
Chin depression 667
Head nodding 667
Head tremor 668
Head thrust 668
HEAD TURN (FACE TURN)
1. Head turned toward right (gaze left)

A. Left Brown syndrome
B. Left inferior oblique muscle palsy
C. Left medial rectus muscle palsy
D. Left superior oblique muscle palsy
E. Right Duane syndrome
F. Right jerk nystagmus
G. Right inferior rectus muscle palsy
H. Right lateral rectus muscle palsy
I. Right superior rectus muscle palsy
J. Right supranuclear gaze paresis
2. Head turned toward left (gaze right)
A. Left Duane syndrome
B. Left jerk nystagmus
C. Left inferior rectus muscle palsy
D. Left lateral rectus muscle palsy
E. Left superior rectus muscle palsy
F. Left supranuclear gaze paresis
G. Right Brown syndrome
H. Right inferior rectus muscle palsy
I. Right medial rectus muscle palsy
J. Right superior oblique muscle palsy
3. Head turned toward either left or right
A. Congenital jerk nystagmus-head turned away from field with least amplitude
of nystagmus (i.e., left jerk nystagmus improves in right gaze; left head turn)
B. Esotropia-head turned in the direction of convergent eye (cross fixation)
C. Hearing defect
D. One blind eye-head turn away affected side (good eye fixates in adduction)
E. Photophobia (see p. 650)
F. Progressive intracranial arterial occlusion syndrome (Taveras syndrome)
G. Strabismus fixus (general fibrosis syndrome)
H. Under corrected myope
Hiatt RL, Cope-Troupe C. Abnormal head positions due to ocular problems. Ann
Ophthalmol 1978; 10:881-892.
Kushner BJ. Ocular causes of abnormal head postures. Ophthalmology 1979; 86:2115-
2125.
Wilkins, 1985.
HEAD TILT (HEAD TILTED TOWARD EITHER SHOULDER OR AROUND AN

ANTEROPOSTERIOR AXIS)
1. Head tilted toward right

A. Left superior oblique muscle palsy
B. Left superior rectus muscle palsy
C. Right inferior oblique muscle palsy
D. Right inferior rectus muscle palsy
2. Head tilted toward left
A. Left inferior oblique muscle palsy
B. Left inferior rectus muscle palsy
C. Right superior oblique muscle palsy
D. Right superior rectus muscle palsy
3. Head tilted toward either right or left
A. Astigmatism
B. Beckwith-Wiedemann syndrome
C. Blowout fracture
D. Incorrectly aligned cylinder axis
E. Monocular torticollis-patching of eyes does not eliminate problem;
roentgenogram may help
(1) Congenital malformation of fracture of cervical spine or vertebral
processes
(2) Fracture of clavicle
(3) Functional habit and hysteria
(4) Pain from infection
a. Adenitis
b. Arthritis
c. Mastoiditis
d. Synovitis
(5) Paralysis of absent muscles on opposite side of head tilt
(6) Sandifer syndrome (hiatus hernia-torticollis syndrome)
(7) Spasm of sternocleidomastoid or contracture of sternocleidomastoid
muscle on side of head tilt
(8) Vestibular defect
a. Acoustic neuroma
b. Labyrinthitis
c. Otitis media
F. Nystagmus-turned away from field with least amplitude of nystagmus
G. Superior oblique tendon sheath syndrome (Brown syndrome)
Greenberg MF, Pollard ZF. Ocular plagiocephaly: ocular torticellis with skull and facial
asymmetry. Ophthalmology 2000; 107:173-179.
Kattah JC, Dagi TF. Compensatory head tilt in upbeating nystagmus. J Clin Neuro-
Ophthalmol 1990; 10:27-31.
Levine RM. Ocular plagiocephaly. Ophthalmology 2000; 107:2123-2124.
Rubin SE, et al. Ocular torticolis. Surv Ophthalmol 1986; 30:366.
CHIN ELEVATION
1. Adaptive symptom of contact lens wearer

2. A-esotropia with fusion in downward gaze
3. Blowout fracture of orbit
4. Brown syndrome (superior oblique tendon sheath syndrome)
5. Double elevator palsy
6. General fibrosis syndrome (strabismus fixus)
7. Incomplete bilateral ptosis
8. Inferior oblique muscle palsy
9. Parinaud syndrome (dorsal midbrain syndrome)
10. Superior rectus muscle palsy
11. Supranuclear lesion (upgaze palsy)
12. Thyroidectomy
13. "V" pattern exotropia with fusion in downward gaze
Beyer-Machule C, von Noorden GK: Atlas of ophthalmic surgery, vol 1: Lids, orbits,
Hiatt RL, Cope-Troupe C. Abnormal head positions due to ocular problems. Ann
Ophthalmol 1978; 10:881-892.
CHIN DEPRESSION
1. "A" pattern exotropia with fusion in upward gaze (A pattern)

2. Inferior rectus muscle palsy
3. Photophobia
4. Progressive supranuclear palsy
5. Superior oblique muscle palsy (bilateral)
6. Supranuclear lesion (down gaze palsy)
7. Uncorrected myope of low degree
8. "V" pattern esotropia with fusion in upward gaze
Beyer-Machule C, von Noorden GK. Atlas of ophthalmic surgery, vol 1: Lids, orbits,
Kushner BJ. Ocular causes of abnormal head postures. Ophthalmology 1979; 86:2115-
2125.
HEAD NODDING
1. Benign or familial tremor

2. Bobble head doll syndrome-to-and-fro bobbing of the head and trunk, at 2- to 3-second
intervals because of cyst of third ventricle
3. Congenital nystagmus
4. Extrapyramidal dysfunction, such as paralysis agitans (Parkinson syndrome)
5. Habit spasm
6. Spasms nutans
Gottlob I, et al. Head nodding is compensatory in spasmus nutans. Ophthalmology 1992;

99:1024-1031.
Rubin SE, Slavin ML. Head nodding associated with intermittent esotropia. J Pediatr
HEAD TREMOR
1. Cerebellar system afflictions (benign essential senile tremor)-most common

2. Extrapyramidal disorder
3. Hereditary postural tremor (familial tremor)
4. Postural tremor
Hughes AJ, et al. Paroxysmal dystonic head tumor. Mov Disord 1991; 6:85-86.
Klawans HL. Rhythmic head tremor. JAMA 1982; 248:1510.
HEAD THRUST
1. Oculomotor apraxia-defect or horizontal voluntary movements

2. Ataxia-telangiectasia syndrome
3. Isolated
4. Male predominance
5. Oral-facial-digital syndrome type II
* = most important

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Ocular Differential Diagnosis

Frederick Hampton Roy, M.D., F.A.C.S.

Published by F.E.P. International

Previous editions: 1972, 1977, 1982, 1987, 1992, 1997, 2002

How to Use This Book xi

I. Regional Signs and Symptoms

II. General Signs and Symptoms

Frederick Hampton Roy, M.D.

Rootman J. Diseases of the orbit. Philadelphia: JB Lippincott, 1988.

Archer KF, et al. Orbital nonchromaffin paraganglioma. Ophthalmology 1989; 96:1659-

Carter K, et al. Ophthalmic manifestations of allergic fungal sinusitis. Am J OphthalmoI

Luxenberg MN. Chloroma. Arch Ophthalmol 1991; 109:734-736.

Lyon DB, et al. Epithelioid hemangioendothelioma of the orbital bones. Ophthalmology

SYNDROMES AND DISEASES ASSOCIATED WITH EXOPHTHALMOS

Henderson JW. Orbital tumors. New York: Thieme-Stratton. 1980.

Wright JE, et al. Orbital venous anomalies. Ophthalmology 1 997; 104:905-913.

Table Exophthalmos (Up to 1 year)

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology 1984; 91:229-

Stasior OG, Roen JL. Traumatic enophthalmos. Ophthalmology 1982; 89:1267.

1. Calcification of more irregular configuration and texture

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Garrity JA, Kennerdell JS. Orbital calcification associated with hemangiopericytoma. Am

Zizmor J, Lombardi G. Atlas of orbital radiology. Birmingham: Aesculapius, 1973.

ORBITAL BRUIT (NOISE HEARD OVER ORBIT WITH STETHOSCOPE)

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Grobb WE, et al. Facial hamartomas in chi1dten: neurofibroma, lymphangioma, and

Kushner FH. Carotid-cavernous fistula as a complication of carotid endarterectomy. Ann

ORBITAL EMPHYSEMA (AIR FOUND IN ORBITAL TISSUES AND ADNEXA

*1. Due to fracture of ethmoid sinuses or orbital floor

Hunts JH, et al Orbital emphysema: staging and acute management. Ophthalmology

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Lanzino G, et al. Orbital pain and unruptured carotid-posterior communicating artery

SHALLOW ORBITS OR DIMINISHED ORBITAL VOLUME (ILLUSION OF

Cursiefen C, et al. Adenoma of the nonpigmented ciliary epithelium mimicking a

Smith DW. Recognizable patterns of human malformation, 4th ed. Philadelphia: WB

PSEUDOHYPERTELORISM (ILLUSION OF INCREASED DISTANCE

DeMyer W. The median cleft face syndrome. Neurology 1967; 17:961.

HYPERTELORISM (INCREASED DISTANCE BETWEEN BONY ORBITS AND

1. Aarskog syndrome (faciodigitogenital syndrome)

HYPOTELORISM (DECREASED DISTANCE BETWEEN BONY ORBITS AND

Evans DG. Dominantly inherited microcephaly, hypotelorism and normal intelligence.

Judisch GF, et al. Orbital hypotelorism. Arch Ophthalmol 1984;102:995-997.

1. Cockayne syndrome (dwarfism with retinal atrophy and deafness)

PROMINENT SUPRAORBITAL RIDGES

1. Apert syndrome (acrocephalosyndactylism syndrome)

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WE

OSTEOLYSIS OF BONY ORBIT

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

Oh KT, et al. Adenocarcinoma of the esophagus presenting as orbital cellulitis. Arch

FOSSA FORMATION OF ORBIT (LOCAL EXPANSION OF BONY ORBITAL

1. Encapsulated benign lacrimal gland tumor

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WE

SUBPERIOSTEAL ORBITAL HEMORRHAGE

1. Generalized diseases with bleeding diatheses

EXPANSION OF ORBITAL MARGINS (USUALLY ASSOCIATED WITH

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

HYPERTROPHY OF ORBITAL BONES (HYPEROSTOSIS OR SCLEROSIS OR

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

EXPANSION OF OPTIC CANAL

1. Increased intracranial pressure

SMALL OPTIC CANALS

Albert DM, Jakobiec FA. Principles and practice of ophthalmology. Philadelphia: WB

EROSION OF OPTIC CANAL